Theearly andlate processing of lysosomal enzymes: Proteolysis and compartmentation

The Metabolic Basis of Inherited Disease.

1988 • 7,933 citations

The Metabolic Basis of Inherited Disease

1990 • 4,327 citations

The Low-Density Lipoprotein Pathway and its Relation to Atherosclerosis

1977 • 2,481 citations

Lysosomes in biology and pathology

1977 • 2,068 citations

The Biogenesis of Lysosomes

1989 • 1,583 citations

Antigen Recognition by Class I-Restricted T Lymphocytes

1989 • 1,207 citations

The Role of Surface Carbohydrates in the Hepatic Recognition and Transport of Circulating Glycoproteins

1974 • 1,200 citations

The Golgi apparatus (complex)-(1954-1981)-from artifact to center stage.

1981 • 990 citations

BIOSYNTHETIC PROTEIN TRANSPORT AND SORTING BY THE ENDOPLASMIC RETICULUM AND GOLGI

1987 • 985 citations

Protein sorting in Saccharomyces cerevisiae: isolation of mutants defective in the delivery and processing of multiple vacuolar hydrolases.

1988 • 977 citations

A Role for a 70-Kilodalton Heat Shock Protein in Lysosomal Degradation of Intracellular Proteins

1989 • 932 citations

LYSOSOMAL ENZYMES AND THEIR RECEPTORS

1986 • 915 citations

Microelectrode studies on the acid microenvironment beneath adherent macrophages and osteoclasts*1

1988 • 887 citations

THE DEVELOPMENT OF NEUTROPHILIC POLYMORPHONUCLEAR LEUKOCYTES IN HUMAN BONE MARROW

1971 • 870 citations

The mannose 6-phosphate receptor and the biogenesis of lysosomes

1988 • 846 citations

Biosynthesis of lysosomal enzymes in fibroblasts. Synthesis as precursors of higher molecular weight.

1980 • 809 citations

Studies on the mechanisms of autophagy: formation of the autophagic vacuole.

1990 • 789 citations

The fungal vacuole: composition, function, and biogenesis

1990 • 758 citations

Lysosomes and storage diseases

1973 • 745 citations

Chloroquine inhibits lysosomal enzyme pinocytosis and enhances lysosomal enzyme secretion by impairing receptor recycling.

1980 • 743 citations

Peptide sequences that target cytosolic proteins for lysosomal proteolysis

1990 • 674 citations

The neutrophil: Function and clinical disorders

1978 • 670 citations

The neutrophil: Function and clinical disorders

1979 • 628 citations

Evidence for receptor-mediated binding of glycoproteins, glycoconjugates, and lysosomal glycosidases by alveolar macrophages.

1978 • 593 citations

The retention signal for soluble proteins of the endoplasmic reticulum

1990 • 531 citations

Trafficking of lysosomal enzymes ¹

1987 • 497 citations

Protein Translocation Across Membranes

1988 • 482 citations

ORIGIN OF GRANULES IN POLYMORPHONUCLEAR LEUKOCYTES

1966 • 476 citations

Mannose 6-Phosphate Receptors and Lysosomal Enzyme Targeting

1989 • 473 citations

Trafficking of lysosomal enzymes in normal and disease states.

1986 • 445 citations

Lysosomes : their role in protein breakdown

1987 • 435 citations

Evidence that luminal ER proteins are sorted from secreted proteins in a post-ER compartment.

1988 • 434 citations

Protein sorting in yeast: Mutants defective in vacuole biogenesis mislocalize vacuolar proteins into the late secretory pathway

1986 • 429 citations

Lysosomes revisited

1983 • 427 citations

Biology of Carbohydrates

1981 • 426 citations

Regulation of Protein Export From the Endoplasmic Reticulum

1988 • 421 citations

Signal sequences

1989 • 417 citations

Biosynthesis of lysosomal enzymes in fibroblasts. Phosphorylation of mannose residues.

1980 • 404 citations

PEP4 gene of Saccharomyces cerevisiae encodes proteinase A, a vacuolar enzyme required for processing of vacuolar precursors.

1986 • 400 citations

The Phosphomannosyl Recognition System for Intracellular and Intercellular Transport of Lysosomal Enzymes

1982 • 379 citations

Co-localization of molecules involved in antigen processing and presentation in an early endocytic compartment

1990 • 377 citations

Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man.

1982 • 364 citations

Studies on the mechanisms of autophagy: maturation of the autophagic vacuole.

1990 • 352 citations

Protein Sorting in Saccharomyces cerevisiae: Isolation of Mutants Defective in the Delivery and Processing of Multiple Vacuolar Hydrolases

1988 • 339 citations

Isolation and characterization of human lysosomal membrane glycoproteins, h-lamp-1 and h-lamp-2. Major sialoglycoproteins carrying polylactosaminoglycan.

1988 • 335 citations

Membrane protein sorting: biosynthesis, transport and processing of yeast vacuolar alkaline phosphatase.

1989 • 330 citations

Saposin proteins: structure, function, and role in human lysosomal storage disorders

1991 • 328 citations

Inherited Disorders of Lysosomal Metabolism

1975 • 322 citations

Cloning and sequence analysis of cDNA for human cathepsin D.

1985 • 318 citations

THE MITOCHONDRIAL PROTEIN IMPORT APPARATUS

1990 • 311 citations

The fungal vacuole: composition, function, and biogenesis.

1990 • 308 citations

Evolution in the structure and function of aspartic proteases

1987 • 298 citations

The PEP4 Gene Encodes an Aspartyl Protease Implicated in the Posttranslational Regulation of Saccharomyces cerevisiae Vacuolar Hydrolases

1986 • 292 citations

Intracellular sorting and processing of a yeast vacuolar hydrolase: proteinase A propeptide contains vacuolar targeting information.

1988 • 287 citations

Accumulation of membrane glycoproteins in lysosomes requires a tyrosine residue at a particular position in the cytoplasmic tail.

1990 • 272 citations

Cloning and expression of steroid sulfatase cDNA and the frequent occurrence of deletions in STS deficiency: Implications for X-Y interchange

1987 • 272 citations

Prelysosomal convergence of autophagic and endocytic pathways

1988 • 272 citations

Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA.

1990 • 271 citations

Perforin-Mediated Target Cell Lysis by Cytolytic T Lymphocytes

1990 • 268 citations

Cathepsin D is membrane-associated in macrophage endosomes.

1988 • 259 citations

Coding of Two Sphingolipid Activator Proteins (SAP-1 and SAP-2) by Same Genetic Locus

1988 • 251 citations

Molecules relevant for T cell‐target cell interaction are present in cytolytic granules of human T lymphocytes

1989 • 251 citations

Liposome-encapsulated antigens are processed in lysosomes, recycled, and presented to T cells

1991 • 250 citations

Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant

1991 • 250 citations

Biosynthesis of lysosomal hydrolases: their synthesis in bound polysomes and the role of co- and post-translational processing in determining their subcellular distribution

1982 • 246 citations

Nucleotide and predicted amino acid sequences of cloned human and mouse preprocathepsin B cDNAs.

1986 • 245 citations

Secretion of lysosomal hydrolases by stimulated and nonstimulated macrophages.

1978 • 241 citations

Proteases and proteolysis in the lysosome

1992 • 240 citations

Control of Proteolysis

1980 • 238 citations

Peptide sequences that target proteins for enhanced degradation during serum withdrawal.

1988 • 238 citations

Biosynthesis and transport of cathepsin D in cultured human fibroblasts.

1983 • 235 citations

Early events in the biosynthesis of the lysosomal enzyme cathepsin D.

1979 • 233 citations

Homology of amino acid sequences of rat liver cathepsins B and H with that of papain.

1983 • 231 citations

Human β-glucuronidase: In vivo clearance and in vitro uptake by a glycoprotein recognition system on reticuloendothelial cells

1978 • 228 citations

DIFFERENCES IN ENZYME CONTENT OF AZUROPHIL AND SPECIFIC GRANULES OF POLYMORPHONUCLEAR LEUKOCYTES

1968 • 227 citations

The discovery of lysosomes.

1981 • 223 citations

Macrophage endosomes contain proteases which degrade endocytosed protein ligands.

1985 • 223 citations

Arylsulfatase A pseudodeficiency: loss of a polyadenylylation signal and N-glycosylation site.

1989 • 219 citations

Expression of cDNA encoding the human “protective protein≓ associated with lysosomal β-galactosidase and neuraminidase: Homology to yeast proteases

1988 • 219 citations

Nonselective autophagy of cytosolic enzymes by isolated rat hepatocytes.

1990 • 218 citations

Cloning and Expression of Human Arylsulfatase A

1989 • 214 citations

Biosynthesis, glycosylation, movement through the Golgi system, and transport to lysosomes by an N-linked carbohydrate-independent mechanism of three lysosomal integral membrane proteins.

1986 • 212 citations

Molecular cloning and nucleotide sequence of human glucocerebrosidase cDNA.

1985 • 209 citations

Lysosomal Cysteine Proteinases

1980 • 206 citations

Mutant defective in processing of an enzyme located in the lysosome-like vacuole of Saccharomyces cerevisiae.

1981 • 199 citations

Human alpha-galactosidase A: nucleotide sequence of a cDNA clone encoding the mature enzyme.

1986 • 198 citations

Kinetics of intracellular transport and sorting of lysosomal membrane and plasma membrane proteins.

1987 • 197 citations

Lysosomal membrane dynamics: structure and interorganellar movement of a major lysosomal membrane glycoprotein.

1986 • 193 citations

The PEP4 gene encodes an aspartyl protease implicated in the posttranslational regulation of Saccharomyces cerevisiae vacuolar hydrolases.

1986 • 192 citations

Human liver cathepsin L

1985 • 191 citations

Lysosomal acid phosphatase is transported to lysosomes via the cell surface.

1989 • 189 citations

Antimicrobial polypeptides of human neutrophils

1990 • 188 citations

Biosynthesis of a lysosomal enzyme. Partial structure of two transient and functionally distinct NH2-terminal sequences in cathepsin D.

1981 • 188 citations

Cloning of cDNAs encoding human lysosomal membrane glycoproteins, h-lamp-1 and h-lamp-2. Comparison of their deduced amino acid sequences.

1988 • 187 citations

Targeting of a lysosomal membrane protein: a tyrosine-containing endocytosis signal in the cytoplasmic tail of lysosomal acid phosphatase is necessary and sufficient for targeting to lysosomes.

1990 • 185 citations

Myeloperoxidase: its structure and expression during myeloid differentiation

1985 • 184 citations

Assembly of storage protein oligomers in the endoplasmic reticulum and processing of the polypeptides in the protein bodies of developing pea cotyledons

1982 • 182 citations

Cloning, sequencing, and expression of cDNA for human beta-glucuronidase.

1987 • 180 citations

Isolation of cDNA clones coding for the alpha-subunit of human beta-hexosaminidase. Extensive homology between the alpha- and beta-subunits and studies on Tay-Sachs disease.

1986 • 176 citations

Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex.

1988 • 174 citations

Regulated import and degradation of a cytosolic protein in the yeast vacuole

1991 • 174 citations

Biosynthesis and molecular cloning of sulfated glycoprotein 1 secreted by rat Sertoli cells: sequence similarity with the 70-kilodalton precursor to sulfatide/GM1 activator

1988 • 166 citations

A peptidase in human platelets that deamidates tachykinins. Probable identity with the lysosomal "protective protein".

1990 • 165 citations

Biosynthesis of the Vacuolar Yeast Glycoprotein Carboxypeptidase Y

1978 • 164 citations

Lysosomal Enzyme Precursors in Human Fibroblasts. Activation of Cathepsin D Precursor in vitro and Activity of beta-Hexosaminidase A Precursor towards Ganglioside GM2

1982 • 163 citations

Epitope-directed processing of specific antigen by B lymphocytes.

1989 • 163 citations

Purification and characterization of human lysosomal membrane glycoproteins

1989 • 163 citations

Human beta-hexosaminidase alpha chain: coding sequence and homology with the beta chain.

1985 • 162 citations

Identification of latent procathepsins B and L in microsomal lumen: Characterization of enzymatic activation and proteolytic processing in vitro

1988 • 161 citations

Processing of human cathepsin D in lysosomes in vitro.

1985 • 159 citations

Natural History and Inherited Disorders of a Lysosomal Enzyme, β-Hexosaminidase

1989 • 157 citations

The lytic granules of natural killer cells are dual-function organelles combining secretory and pre-lysosomal compartments.

1990 • 152 citations

Antimicrobial polypeptides of human neutrophils

1990 • 152 citations

Overproduction-induced mislocalization of a yeast vacuolar protein allows isolation of its structural gene.

1986 • 151 citations

Binding and Uptake of Transcobalamin II by Human Fibroblasts

1978 • 148 citations

The major excreted protein of transformed fibroblasts is an activable acid-protease.

1986 • 147 citations

Nucleotide sequence of the human α-galactosidase A gene

1989 • 146 citations

SEC7 encodes an unusual, high molecular weight protein required for membrane traffic from the yeast Golgi apparatus.

1988 • 145 citations

Generation of a lysosomal enzyme targeting signal in the secretory protein pepsinogen

1990 • 144 citations

Isolation and characterization of a steroid sulfatase cDNA clone: genomic deletions in patients with X-chromosome-linked ichthyosis.

1987 • 143 citations

Biosynthesis of the lysosomal enzyme glucocerebrosidase.

1985 • 139 citations

Dexamethasone increases expression of mannose receptors and decreases extracellular lysosomal enzyme accumulation in macrophages.

1985 • 139 citations

Cloning and characterization of a mouse cysteine proteinase.

1986 • 137 citations

The post-translational proteolysis of the subunits of vicilin from pea (Pisum sativum L.)

1982 • 136 citations

Complete nucleotide and deduced amino acid sequences of human and murine preprocathepsin L. An abundant transcript induced by transformation of fibroblasts.

1988 • 133 citations

Characterisation and Subunit Structures of the Vicilin Storage Proteins of Pea (Pisum sativum L.)

1981 • 133 citations

Spatial conformation of glycans and glycoproteins

1984 • 132 citations

Role of the endoplasmic reticulum in the synthesis of reserve proteins and the kinetics of their transport to protein bodies in developing pea cotyledons.

1982 • 129 citations

Nucleotide sequence of cDNA containing the complete coding sequence for human lysosomal glucocerebrosidase.

1986 • 129 citations

Human lysosomal acid phosphatase is transported as a transmembrane protein to lysosomes in transfected baby hamster kidney cells.

1988 • 129 citations

Immunologic Abnormalities in Subacute Sclerosing Panencephalitis

1971 • 128 citations

Biosynthesis of lysosomal endopeptidases

1989 • 128 citations

Organization of the gene encoding the human beta-hexosaminidase alpha-chain.

1987 • 127 citations

Structure of the N-asparagine-linked oligosaccharide units of human placental beta-glucocerebrosidase.

1984 • 125 citations

A novel pathway of import of alpha-mannosidase, a marker enzyme of vacuolar membrane, in Saccharomyces cerevisiae.

1990 • 125 citations

Proteolytic activities in yeast

1975 • 124 citations

Antigen processing and intracellular Ia. Possible roles of endocytosis and protein synthesis in Ia function.

1989 • 122 citations

Biosyntheses and processing of lysosomal cysteine proteinases in rat macrophages

1988 • 121 citations

Intracellular Sorting and Processing of a Yeast Vacuolar Hydrolase: Proteinase A Propeptide Contains Vacuolar Targeting Information

1988 • 121 citations

Cloning of a cDNA for steroid sulfatase: frequent occurrence of gene deletions in patients with recessive X chromosome-linked ichthyosis.

1987 • 117 citations

Isolation of cDNA clones coding for the beta subunit of human beta-hexosaminidase.

1985 • 116 citations

Gene encoding the human beta-hexosaminidase beta chain: extensive homology of intron placement in the alpha- and beta-chain genes.

1988 • 115 citations

Molecular cloning and sequencing of cDNA for rat cathepsin L

1987 • 115 citations

Secretion of sphingolipid hydrolase activator precursor, prosaposin

1991 • 114 citations

Cloning and analysis of cDNAs encoding plant storage protein precursors

1982 • 114 citations

The Precursor of Sulfatide Activator Protein is Processed to Three Different Proteins

1988 • 114 citations

Synthesis of beta-hexosaminidase in cell-free translation and in intact fibroblasts: an insoluble precursor alpha chain in a rare form of Tay-Sachs disease.

1982 • 113 citations

Mitochondrial import receptors for precursor proteins

1991 • 112 citations

Defects in synthesis, phosphorylation, and maturation of acid alpha-glucosidase in glycogenosis type II.

1985 • 111 citations

Cloning and Expression of Human Steroid-sulfatase

1989 • 110 citations

Human cathepsin B1. Inhibition by α2-macroglobulin and other serum proteins

1973 • 107 citations

Human lysosomal acid phosphatase: cloning, expression and chromosomal assignment.

1988 • 106 citations

Biosynthesis of cathepsin B in cultured normal and I-cell fibroblasts.

1987 • 104 citations

Biosynthesis of two lysosomal enzymes in macrophages. Evidence for a precursor of beta-galactosidase.

1979 • 101 citations

Myeloperoxidase biosynthesis by a human promyelocytic leukemia cell line: insight into myeloperoxidase deficiency

1986 • 98 citations

Oligosaccharides in Lysosomal Enzymes

1981 • 97 citations

Identification and in Vitro Reconstitution of Lysosomal Neuraminidase from Human Placenta

1989 • 96 citations

Identification and characterization of LAMP-1 as an activation-dependent platelet surface glycoprotein.

1990 • 95 citations

Carboxyl-terminal proteolytic processing during biosynthesis of the lysosomal enzymes .beta.-glucuronidase and cathepsin D

1983 • 95 citations

PEP4 Gene of Saccharomyces cerevisiae Encodes Proteinase A, a Vacuolar Enzyme Required for Processing of Vacuolar Precursors

1986 • 94 citations

Effect of proteinase inhibitors on intracellular processing of cathepsin B, H and L in rat macrophages

1988 • 94 citations

Biosynthesis and maturation of glucocerebrosidase in Gaucher fibroblasts

1987 • 94 citations

Structures at the proteolytic processing region of cathepsin D.

1988 • 93 citations

A Frameshift Mutation in a Patient with Tay-Sachs Disease Causes Premature Termination and Defective Intracellular Transport of the α-Subunit of β-Hexosaminidase

1989 • 92 citations

Abnormal lysosomal hydrolases excreted by cultured fibroblasts in I-cell disease (mucolipidosis II)

1975 • 92 citations

In vivo biosynthesis of the vacuolar proteinases A and B in the yeast Saccharomyces cerevisiae.

1982 • 92 citations

Precursors of ricin and Ricinus communis agglutinin

1985 • 92 citations

Enzymic Content of the Mitochondria Fraction

1953 • 91 citations

The biosynthesis and processing of high molecular weight precursors of soybean glycinin subunits.

1982 • 91 citations

Cathepsin B-related proteases in the insulin secretory granule.

1984 • 89 citations

Processing and lysosomal localization of a glycoprotein whose secretion is transformation stimulated.

1985 • 89 citations

Serum and urine in diabetics on diagnosis and subsequent treatment, and stable insulin dependent diabetics

1979 • 87 citations

Amino acid sequences of the human kidney cathepsins H and L

1988 • 87 citations

Structural characteristics of the PHO8 gene encoding repressible alkaline phosphatase in Saccharomyces cerevisiae

1987 • 86 citations

UDP-N-acetylglucosamine:lysosomal enzyme precursor N-acetylglucosamine-1-phosphotransferase. Partial purification and characterization of the rat liver Golgi enzyme.

1982 • 85 citations

Maturation of vacuolar (lysosomal) enzymes in yeast: proteinase yscA and proteinase yscB are catalysts of the processing and activation event of carboxypeptidase yscY.

1987 • 85 citations

Isolation and characterization of a mannose-specific endocytosis receptor from human placenta.

1987 • 85 citations

Mutations in PEP4 locus of Saccharomyces cerevisiae block final step in maturation of two vacuolar hydrolases.

1983 • 84 citations

Internalization of cytoplasmic protein by hepatic lysosomes in basal and deprivation-induced proteolytic states.

1981 • 84 citations

Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A (cerebroside sulfatase) activity by inhibitors of thiol proteinases.

1983 • 83 citations

Homology of the precursor of pulmonary surfactant-associated protein SP-B with prosaposin and sulfated glycoprotein 1.

1991 • 83 citations

Interrelationship of active and latent secreted human cathepsin B precursors

1986 • 82 citations

Isolation and characterization of a mannose-specific endocytosis receptor from rabbit alveolar macrophages

1987 • 82 citations

Protein targeting to the yeast vacuole

1989 • 81 citations

Processing pathway for protease B of Saccharomyces cerevisiae.

1989 • 81 citations

The role of extra-hepatic tissues in the receptor-mediated plasma clearance of glycoproteins terminated by mannose or N-acetylglucosamine

1980 • 81 citations

Synthesis and processing of cathepsin L, an elastase, by human alveolar macrophages

1989 • 79 citations

Proteolytic processing of pro-alpha and pro-beta precursors from human beta-hexosaminidase. Generation of the mature alpha and beta a beta b subunits.

1988 • 79 citations

Molecular cloning of a cDNA for human alpha-L-fucosidase.

1985 • 78 citations

A new class of lysosomal/vacuolar protein sorting signals.

1990 • 77 citations

Mouse "protective protein". cDNA cloning, sequence comparison, and expression.

1990 • 77 citations

Distribution of saposin proteins (sphingolipid activator proteins) in lysosomal storage and other diseases.

1990 • 76 citations

Excretion-Reuptake Route of β-Hexosaminidase in Normal and I-Cell Disease Cultured Fibroblasts

1979 • 76 citations

Sequential processing of lysosomal acid phosphatase by a cytoplasmic thiol proteinase and a lysosomal aspartyl proteinase.

1989 • 76 citations

Protein transport across the ER membrane

1990 • 75 citations

Myeloperoxidase is synthesized as larger phosphorylated precursor.

1984 • 74 citations

LAMP-1 in cho cells is a primary carrier of poly-N-acetyllactosamine chains and is bound preferentially by a mammalian S-type lectin

1990 • 73 citations

Identification of cDNA clones encoding a precursor of rat liver cathepsin B.

1985 • 73 citations

Amino acid sequence of porcine spleen cathepsin D.

1984 • 73 citations

Human liver glucuronate 2-sulphatase. Purification, characterization and catalytic properties

1989 • 72 citations

The selective release of lysosomal acid hydrolases from mouse peritoneal macrophages by stimuli of chronic inflammation.

1977 • 71 citations

Evidence that aspartic proteinase is involved in the proteolytic processing event of procathepsin L in lysosomes

1989 • 70 citations

Pathways involved in targeting and secretion of a lysosomal enzyme in Dictyostelium discoideum.

1985 • 70 citations

Mannose 6-phosphate receptors and their role in targeting proteins to lysosomes

1988 • 69 citations

Biosynthesis, transport and processing of myeloperoxidase in the human leukaemic promyelocytic cell line HL-60 and normal marrow cells

1984 • 69 citations

Oligosaccharide units of lysosomal cathepsin D from porcine spleen. Amino acid sequence and carbohydrate structure of the glycopeptides.

1983 • 68 citations

Posttranslational processing of a human myeloid lysosomal protein, myeloperoxidase

1987 • 66 citations

Glucocerebrosidase, a lysosomal enzyme that does not undergo oligosaccharide phosphorylation

1988 • 66 citations

Human alpha-N-acetylgalactosaminidase-molecular cloning, nucleotide sequence, and expression of a full-length cDNA. Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene.

1990 • 66 citations

Synthesis and Processing of Arylsulfatase A in Human Skin Fibroblasts

1982 • 65 citations

Limited proteolysis of the beta-hexosaminidase precursor in a cell-free system.

1981 • 64 citations

Cloning and expression of the gene for the major excreted protein of transformed mouse fibroblasts. A secreted lysosomal protease regulated by transformation.

1988 • 64 citations

Isolation of procathepsin D from mature cathepsin D by pepstatin affinity chromatography. Autocatalytic proteolysis of the zymogen form of the enzyme

1989 • 64 citations

Proteolytic processing of the alpha-chain of the lysosomal enzyme, beta-hexosaminidase, in normal human fibroblasts.

1988 • 63 citations

Faulty association of alpha- and beta-subunits in some forms of beta-hexosaminidase A deficiency.

1984 • 62 citations

Turnover of two lysosomal enzymes in macrophages.

1981 • 62 citations

Effects of cysteine protease inhibitors on rabbit cathepsin D maturation

1989 • 62 citations

Nucleotide sequence of rat preputial gland beta-glucuronidase cDNA and in vitro insertion of its encoded polypeptide into microsomal membranes.

1986 • 61 citations

Processing of a newly identified intermediate of human myeloperoxidase in isolated granules occurs at neutral pH.

1986 • 61 citations

Synthesis and processing of sphingolipid activator protein-2 (SAP-2) in cultured human fibroblasts.

1986 • 59 citations

The Biochemical Basis of Gangliosidoses

1984 • 59 citations

Human liver iduronate-2-sulphatase. Purification, characterization and catalytic properties

1990 • 59 citations

Novel carbohydrate structures of cathepsin B from porcine spleen.

1984 • 58 citations

The ligand binding specificity and tissue localization of a rat alveolar macrophage lectin.

1986 • 58 citations

Isolation of a cDNA encoding the human G_M2 activator protein

1989 • 58 citations

The complete amino‐acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein

1990 • 58 citations

Biosynthesis and transport of lysosomal enzymes in human monocytes and macrophages. Effects of ammonium chloride, zymosan and tunicamycin

1983 • 58 citations

Neutrophil stimulation: receptor, membrane, and metabolic events.

1984 • 57 citations

Analysis of the glycosylation and phosphorylation of the lysosomal enzyme, β-hexosaminidase B, by site-directed mutagenesis

1989 • 56 citations

Human β-GIucuronidase. II. Fate of Infused Human Placental β-Glucuronidase in the Rat

1977 • 56 citations

Primary amines induce selective release of lysosomal enzymes from mouse macrophages

1980 • 56 citations

Myeloperoxidase precursors in human myeloid leukemia HL-60 cells.

1982 • 56 citations

Subcellular location and properties of bactericidal factors from human neutrophils.

1986 • 55 citations

Biogenesis of the yeast lysosome (vacuole): biosynthesis and maturation of proteinase yscB.

1988 • 55 citations

The activator protein for glucosylceramide beta-glucosidase from guinea pig liver. Improved isolation method and complete amino acid sequence.

1988 • 55 citations

Biosynthesis and processing of lysosomal cathepsin L in primary cultures of rat hepatocytes

1988 • 55 citations

Effect of monensin on intracellular transport and receptor-mediated endocytosis of lysosomal enzymes

1984 • 54 citations

Enhanced proteolytic degradation of normal β-galactosidase in the lysosomal storage disease with combined β-galactosidase and neuraminidase deficiency

1982 • 54 citations

Deficient lysosomal carboxypeptidase activity in galactosialidosis

1990 • 54 citations

Characteristics ofN-formyl-methionyl-leucyl-phenylalanine as an inducer of lysosomal enzyme release from human neutrophils

1980 • 54 citations

Structure of the lysosomal neuraminidase–β-galactosidase–carboxypeptidase multienzymic complex

1990 • 53 citations

Molecular forms of β-hexosaminidase and cathepsin D in serum and urine of healthy subjects and patients with elevated activity of lysosomal enzymes

1983 • 53 citations

Identification of a 31,500 molecular weight islet cell protease as cathepsin B.

1983 • 52 citations

Porcine spleen cathepsin B is an exopeptidase.

1986 • 52 citations

Complete Amino-Acid Sequence and Carbohydrate Content of the Naturally Occurring Glucosylceramide Activator Protein (A₁Activator) Absent from a New Human Gaucher Disease Variant

1987 • 52 citations

The secretory code of the neutrophil.

1979 • 51 citations

Properties of N-acetyl-beta-D-hexosaminidase from isolated normal and I-cell lysosomes.

1981 • 49 citations

The uptake of enzymes into lysosomes: an overview.

1980 • 49 citations

Two mutations produce intron insertion in mRNA and elongated beta-subunit of human beta-hexosaminidase.

1990 • 48 citations

Processing of human beta-galactosidase in GM1-gangliosidosis and Morquio B syndrome.

1984 • 48 citations

Inhibitor studies indicate that active cathepsin L is probably essential to its own processing in cultured fibroblasts

1990 • 48 citations

Isolation of a cDNA clone for the human lysosomal proteinase cathepsin B.

1986 • 48 citations

Processing and secretion of alpha-mannosidase forms by Dictyostelium discoideum.

1982 • 48 citations

Lysosomal enzymes in Dictyostelium discoideum are transported to lysosomes at distinctly different rates.

1986 • 47 citations

Turnover of β-galactosidase in fibroblasts from patients with genetically different types of β-galactosidase deficiency

1981 • 46 citations

Enhanced degradation of cathepsin D synthesized in the presence of the threonine analog β-hydroxynorvaline

1984 • 46 citations

Recognition of human urine α-N-acetylglucosaminidase by rat hepatocytes. Involvement of receptors specific for galactose, mannose 6-phosphate and mannose

1979 • 45 citations

Biosynthesis of lysosomal cathepsins B and H in cultured rat hepatocytes

1988 • 45 citations

Human α-L-fucosidase: Complete coding sequence form cDNA clones

1989 • 45 citations

Subcellular redistribution of newly synthesized macrophage lysosomal enzymes. Correlation between delivery to the lysosomes and maturation.

1983 • 44 citations

Structural studies of the endoglycosidase H-resistant oligosaccharides present on human beta-glucuronidase.

1982 • 44 citations

Intracellular transport and processing of lysosomal cathepsin B

1987 • 44 citations

The processing and intracellular transport of myeloperoxidase. Modulation by lysosomotropic agents and monensin.

1986 • 43 citations

Molecular Cloning and Sequencing of a cDNA Coding for Mature Human Kidney Cathepsin H

1988 • 42 citations

Synthesis and assembly of a catalytically active lysosomal enzyme, beta-hexosaminidase B, in a cell-free system.

1988 • 42 citations

Structural Studies on the Carbohydrate Moieties of Rat Liver Cathepsins B and H1

1985 • 41 citations

Nucleotide sequence of cloned cDNA for human sphingolipid activator protein 1 precursor.

1987 • 41 citations

The role of the cathepsin D propeptide in sorting to the lysosome.

1992 • 41 citations

Uptake and apparent digestion of cytoplasmic organelles by protein bodies (protein storage vacuoles) in mung bean cotyledons.

1981 • 40 citations

Porcine spleen cathepsin H hydrolyzes oligopeptides solely by aminopeptidase activity.

1988 • 40 citations

Proteolytic Processing of the β-Subunit of the Lysosomal Enzyme, β-Hexosaminidase, in Normal Human Fibroblasts

1989 • 39 citations

Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human .beta.-hexosaminidase

1988 • 38 citations

Nucleotide sequence of human preprocathepsin H, a lysosomal cysteine proteinase

1989 • 38 citations

Reconstitution of Endosomal Proteolysis in a Cell-free System

1989 • 38 citations

β-Galactosidase-Neuraminidase Deficiency: Restoration of β-Galactosidase Activity by Protease Inhibitors1

1981 • 37 citations

Gene structure and 5'‐upstream sequence of rat cathepsin L

1989 • 37 citations

The activation of resting lymphocytes is accompanied by the biogenesis of lysosomal organelles

1990 • 36 citations

Mannose-receptor ligands stimulate secretion of lysosomal enzymes from rabbit alveolar macrophages.

1987 • 36 citations

Biochemical Analysis of the Movement of a Major Lysosomal Membrane Glycoprotein in the Endocytic Membrane System

1989 • 36 citations

Identification of a precursor form of cathepsin D in microsomal lumen: Characterization of enzymatic activation and proteolytic processing

1987 • 35 citations

Intracellular maturation and secretion of acid phosphatase of Saccharomyces cerevisiae

1985 • 35 citations

Secretion and uptake of ß-N-acetylglucosaminidase by fibroblasts Effect of chloroquine and mannose 6-phosphate

1979 • 35 citations

Processing of a plant vacuolar protein precursor in vitro

1987 • 35 citations

Human Cathepsin D

1977 • 34 citations

Genetic dissection of the early stages of protein secretion in yeast

1989 • 34 citations

Synthesis and processing of in vitro and in vivo precursors of the vacuolar yeast enzyme carboxypeptidase Y.

1981 • 34 citations

Comparative studies of two cathepsin B isozymes from porcine spleen. Isolation, polypeptide chain arrangements, and enzyme specificity.

1986 • 33 citations

[Intracellular processing and activation of lysosomal cathepsins].

1988 • 33 citations

In vitro translation and processing of cathepsin B of Schistosoma mansoni.

1990 • 33 citations

The processing of a cathepsin L precursor in vitro

1989 • 33 citations

Targeting of beta-glucuronidase to lysosomes in mannose 6-phosphate receptor-deficient MOPC 315 cells.

1984 • 33 citations

Expression and maturation of human cathepsin D in baby-hamster kidney cells

1991 • 32 citations

Cellular and molecular events in spermiogenesis

1991 • 32 citations

Isolation and sequence analysis of a cDNA encoding rat liver α-<scp>l</scp>-fucosidase

1989 • 32 citations

Diagnostic significance of some urinary enzymes for detecting acute rejection crises in renal-transplant recipients: alanine aminopeptidase, alkaline phosphatase, gamma-glutamyltransferase, N-acetyl-beta-D-glucosaminidase, and lysozyme.

1986 • 32 citations

Endocytosis of lysosomal enzymes by non-parenchymal rat liver cells. Comparative study of lysosomal-enzyme uptake by hepatocytes and non-parenchymal liver cells

1979 • 32 citations

An immunocytochemical study on co-localization of cathepsin B and atrial natriuretic peptides in secretory granules of atrial myoendocrine cells of rat heart.

1989 • 31 citations

The synthesis and processing of β-glucuronidase in normal and egasyn deficient mouse kidney

1981 • 31 citations

Comparative studies of asparagine-linked oligosaccharide structures of rat liver microsomal and lysosomal β-glucuronidases

1981 • 31 citations

Intracellular processing and activation of lysosomal cathepsins.

1990 • 31 citations

Isolation and sequencing of a cDNA clone encoding acid phosphatase in rat liver lysosomes

1989 • 31 citations

Enzymatically active cathepsin B dissociating from its inhibitor complexes is elevated in blood plasma of patients with septic shock and some malignant tumors.

1988 • 30 citations

Processing, transport, and secretion of the lysosomal enzyme acid phosphatase in Dictyostelium discoideum

1989 • 30 citations

Molecular cloning of a full-length cDNA for human α-N-acetylgalactosaminidase (α-galactosidase B)

1989 • 30 citations

Amino acid sequence of bovine spleen cathepsin B.

1988 • 30 citations

Autodegradation of lysosomal cysteine proteinases

1987 • 30 citations

Gene structure of rat cathepsin H

1989 • 30 citations

The amino-terminal sequences in the pro-α and -β polypeptides of human lysosomal β-hexosaminidase A and B are retained in the mature isozymes

1989 • 30 citations

Identification of latent procathepsin H in microsomal lumen: Characterization of proteolytic processing and enzyme activation

1988 • 30 citations

Amino acid sequence of porcine spleen cathepsin D light chain.

1983 • 29 citations

Structural and immunochemical characterization of human urine arylsulfatase A purified by affinity chromatography

1985 • 29 citations

Differential segregation of human and hamster cathepsin D in transfected baby-hàmster kidney cells

1991 • 29 citations

Cathepsinogen D: characterization and activation to cathepsin D and inhibitory peptides

1981 • 28 citations

Immunoassay of β-hexosaminidase isoenzymes in serum in patients with raised total activities

1989 • 28 citations

Synthesis and maturation of the yeast vacuolar enzymes carboxypeptidase Y and aminopeptidase I

1983 • 28 citations

GMl-gangliosidosis: Abnormalities in biosynthesis and early processing of β-galactosidase in fibroblasts

1988 • 27 citations

Storage protein precursor polypeptides in cotyledons of Pisum sativum L.. Identification of, and isolation of a cDNA clone for, an 80000-Mr legumin-related polypeptide

1984 • 27 citations

Inhibition of aspartic proteinases by α2-macroglobulin

1989 • 27 citations

Amino acid sequence of chicken liver cathepsin L

1987 • 26 citations

Inhibition by cyanate of the processing of lysosomal enzymes

1983 • 26 citations

Biosynthesis of carboxypeptidase Y in yeast. Evidence for a precursor form of the glycoprotein

1976 • 26 citations

Rat liver β-glucuronidase. cDNA cloning, sequence comparisons and expression of a chimeric protein in COS cells

1988 • 25 citations

Translocation of a lysosomal enzyme across the microsomal membrane requires signal recognition particle

1983 • 25 citations

[29] Measurement of secretion of lysosomal acid glycosidases

1989 • 25 citations

Sulfated N-linked oligosaccharides affect secretion but are not essential for the transport, proteolytic processing, and sorting of lysosomal enzymes in Dictyostelium discoideum.

1990 • 25 citations

Molecular defect in processing α-fucosidase in fucosidosis

1985 • 24 citations

Structural studies of rat cathepsin E: Amino-terminal structure and carbohydrate units of mature enzyme

1990 • 24 citations

Two allelic forms of human arylsulfatase A with different numbers of asparagine-linked oligosaccharides.

1983 • 24 citations

Determination of the structure of the carbohydrate chains of acid α-glucosidase from human placenta

1987 • 23 citations

Nonhuman cells correctly sort and process the human lysosomal enzyme cathepsin D

1989 • 23 citations

Sulfated oligosaccharides in human lysosomal enzymes

1987 • 23 citations

Distinct chromatographic forms of human hemi-myeloperoxidase obtained by reductive cleavage of the dimeric enzyme. Evidence for subunit heterogeneity.

1990 • 23 citations

Molecular Forms of G_M2-Activator Protein. A Study on its Biosynthesis in Human Skin Fibroblasts

1985 • 23 citations

Structure and function of proparts in zymogens for aspartic proteinases.

1988 • 22 citations

Structural analysis of the carbohydrate chains of β-N-acetylhexosaminidases from bovine brain

1985 • 22 citations

Cell contact induces the synthesis of a lysosomal enzyme precursor in lymphocytes and its direct transfer to fibroblasts

1988 • 22 citations

Activation mechanism of monkey and porcine pepsinogens A. One-step and stepwise activation pathways and their relation to intramolecular and intermolecular reactions

1987 • 22 citations

Oligosaccharides on cathepsin D from porcine spleen

1984 • 22 citations

Limited proteolysis of rabbit cardiac procathepsin D in a cell-free system

1986 • 22 citations

Multiple sulfatase deficiency: Degradation of arylsulfatase A and B after endocytosis in fibroblasts

1985 • 21 citations

Inhibition of intracellular sorting and processing of lysosomal cathepsins H and L at reduced temperature in primary cultures of rat hepatocytes

1990 • 20 citations

Localization of the pro‐sequence within the total deduced primary structure of human β‐hexosaminidase B

1988 • 20 citations

Processing and Transport of Lysosomal Enzymes in Human Monocyte Line U937

1984 • 20 citations

Biosynthesis and processing of legumin-like storage proteins in Lupinus angustifolius (lupin)

1985 • 20 citations

Association of the precursor of cathepsin D with coated membranes. Kinetics and carbohydrate processing

1987 • 20 citations

Heterogeneity in late-onset metachromatic leukodystrophy. Effect of inhibitors of cysteine proteinases.

1986 • 19 citations

Characterization of β-Glucosidase as a Peripheral Enzyme of Lysosomal Membranes from Mouse Liver and Purification

1985 • 19 citations

Lysosomal integral membrane glycoproteins are expressed at high levels in the inclusion bodies of I-cell disease fibroblasts

1989 • 18 citations

In vitro biosynthesis of the lysosomal cathepsin H

1987 • 17 citations

Identification of Monkey Lung Procathepsin <scp>d</scp>‐II as a Pepsinogen‐C‐Like Acid Protease Zymogen

1983 • 17 citations

Evidence for the involvement of an acidic compartment in the processing of myeloperoxidase in human promyelocytic leukemia HL-60 cells

1987 • 17 citations

Proteolytic Processing in Endosomal Vesicles

1989 • 17 citations

Evidence for non-vacuolar proteolytic catabolite inactivation of yeast fructose-1,6-bisphosphatase

1987 • 17 citations

Intracellular transport and processing of lysosomal cathepsin H

1987 • 17 citations

Human neuraminidase is a 60 kDa-processing product of prosaposin

1990 • 17 citations

Transport and processing of endocytosed lysosomal alpha-glucosidase in cultured human skin fibroblasts

1986 • 16 citations

Targeting of Lysosomal Acid Phosphatase with Altered Carbohydrate

1989 • 16 citations

Hexosaminidase a deficient adults: Presence of α chain precursor in cultured skin fibroblasts

1984 • 15 citations

Human sphingolipid activator protein-1 and sphingolipid activator protein-2 are encoded by the same gene.

1989 • 15 citations

Subunit Structures of Three Human Myeloperoxidases1

1988 • 15 citations

Structural studies on the carbohydrate moieties of human liver α-l-fucosidase

1988 • 15 citations

Low Temperature Blocks Transport and Sorting of Cathepsin D in Fibroblasts

1988 • 15 citations

Lack of proteolytic processing of α‐L‐fucosidase in human skin fibroblasts

1988 • 15 citations

Effect of the co-existence of galactosyl and phosphomannosyl residues on β-hexosaminidase on the processing and transport of the enzyme in mucolipidosis I fibroblasts

1983 • 14 citations

Biosynthesis of secreted β-hexosaminidase in Tetrahymena thermophila. A comparison of the wild type with a secretory mutant

1988 • 14 citations

Lymphocytes transfer only the lysosomal form of α-d-mannosidase during cell-to-cell contact

1988 • 13 citations

Brefeldin A Prevents Uncovering but not Phosphorylation of the Recognition Marker in Cathepsin D

1990 • 13 citations

Transport of acid phosphatase to lysosomes does not involve passage through the cell surface

1990 • 13 citations

Lysosomal acid phosphatase is transported via endosomes to lysosomes

1990 • 12 citations

Isoenzymes of N-Acetyl-β-Glucosaminidase frorn Human Liver and Serum: Separation by Electrofocusing in Thin Layers of Polyacrylamide Gel

1978 • 12 citations

Abnormal expression of?-L-fucosidase in lymphoid cell lines of fucosidosis patients

1989 • 12 citations

Phosphorylation and sulfation of arylsulfatase A accompanies biosynthesis of the enzyme in normal and carcinoma cell lines

1985 • 11 citations

Impaired proteolytic processing of lysosomal N-acetyl-β-Hexosaminidase in cultured fibroblasts from patients with infantile generalized N-acetylneuraminic acid storage disease

1988 • 11 citations

RABBIT LIVER β-GLUCURONIDASE

1971 • 10 citations

The primary structure of cathepsin D and the implications for its biological functions

1985 • 9 citations

Biosynthesis of human α-N-acetylgalactosaminidase: Defective phosphorylation and maturation in infantile α-NAGA deficiency

1991 • 9 citations

isoenzymes by chromatofocusing from serum and skin in diabetes

1984 • 9 citations

Historical Fragments; Methods; Some Terminology

1989 • 8 citations

LYSOSOMAL STORAGE DISORDERS CAUSED BY INSTABILITY OF THE MISSING ENZYMES

1984 • 8 citations

Purification and characterization of two different precursor forms of the cathepsin B-like proteinase from human malignant ascitic fluid.

1988 • 7 citations

Isolation and partial characterization of the oligosaccharide moieties of rat preputial β-glucuronidase

1981 • 7 citations

Lysosomal Membrane Proteins do not Bind to Mannose-6-Phosphate-Specific Receptors

1986 • 7 citations

CLONING OF LYSOSOMAL GENES

1984 • 6 citations

The Mannose Recognition Pathway-Implications for Lysosome Physiology

1984 • 5 citations

α2-macroglobulin used to isolate intracellular endopeptidases from mammalian cells in culture

1988 • 5 citations

CHROMOSOMAL LOCALIZATION OF THE STRUCTURAL GENES ENCODING THE HUMAN LYSOSOMAL HYDROLASES AND THEIR ACTIVATOR AND STABILIZER PROTEINS

1984 • 5 citations

Changes in the Catalytic Activities of Proteoglycan-Degrading Lysosomal Enzymes in Parenchymal and Non-Parenchymal Liver Cells and in Serum During the Development of Experimental Liver Fibrosis

1983 • 4 citations