The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

The Metabolic and Molecular Bases of Inherited Disease

1995 • 12,086 citations

Functional rafts in cell membranes

1997 • 9,471 citations

Niemann-Pick C1 Disease Gene: Homology to Mediators of Cholesterol Homeostasis

1997 • 1,440 citations

Murine Model of Niemann-Pick C Disease: Mutation in a Cholesterol Homeostasis Gene

1997 • 797 citations

Multivesicular endosomes containing internalized EGF-EGF receptor complexes mature and then fuse directly with lysosomes.

1996 • 531 citations

Identification of two lysosomal membrane glycoproteins.

1985 • 475 citations

Low temperature selectively inhibits fusion between pinocytic vesicles and lysosomes during heterophagy of 125I-asialofetuin by the perfused rat liver.

1980 • 451 citations

Intracellular Trafficking of Cholesterol Monitored with a Cyclodextrin

1996 • 366 citations

Adsorptive endocytosis of Semliki Forest virus

1980 • 354 citations

A quantitative analysis of the endocytic pathway in baby hamster kidney cells.

1989 • 315 citations

The intracellular transport of low density lipoprotein-derived cholesterol is inhibited in Chinese hamster ovary cells cultured with 3-β-[2-(diethylamino)ethoxy]androst-5-en-17-one

1989 • 315 citations

Cholesterol-dependent retention of GPI-anchored proteins in endosomes

1998 • 313 citations

The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts.

1989 • 306 citations

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

1991 • 250 citations

Cycling of the integral membrane glycoprotein, LEP100, between plasma membrane and lysosomes: Kinetic and morphological analysis

1987 • 244 citations

Dense core lysosomes can fuse with late endosomes and are re-formed from the resultant hybrid organelles

1997 • 234 citations

Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol.

1988 • 230 citations

Fusion of Lysosomes with Late Endosomes Produces a Hybrid Organelle of Intermediate Density and Is NSF Dependent

1998 • 216 citations

Exocytosis of pinocytosed fluid in cultured cells: kinetic evidence for rapid turnover and compartmentation.

1981 • 215 citations

Quantification of low density lipoprotein and transferrin endocytic sorting in HEp2 cells using confocal microscopy

1994 • 212 citations

Type-C Niemann-Pick disease: low density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes.

1988 • 203 citations

Group C Niemann‐Pick disease: faulty regulation of low‐density lipoprotein uptake and cholesterol storage in cultured fibroblasts

1987 • 196 citations

Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature.

1999 • 195 citations

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

1986 • 193 citations

Progesterone blocks cholesterol translocation from lysosomes.

1992 • 170 citations

Topology of glycosphingolipid degradation

1996 • 162 citations

Evidence for a Cholesterol Transport Pathway from Lysosomes to Endoplasmic Reticulum That Is Independent of the Plasma Membrane

1998 • 160 citations

The biogenesis of lysosomes: Is it a kiss and run, continuous fusion and fission process?

1996 • 144 citations

Differential accumulation of cholesterol in Golgi compartments of normal and Niemann-Pick type C fibroblasts incubated with LDL: a cytochemical freeze-fracture study

1993 • 135 citations

AP-2-containing clathrin coats assemble on mature lysosomes.

1996 • 132 citations

Delivery to lysosomes in the human carcinoma cell line HEp-2 involves an actin filament-facilitated fusion between mature endosomes and preexisting lysosomes.

1995 • 122 citations

Evidence for retrograde traffic between terminal lysosomes and the prelysosomal/late endosome compartment

1994 • 117 citations

Niemann–Pick disease type C

1998 • 108 citations

Physiological functions of endosomal proteolysis

1995 • 91 citations

Growth of Ectopic Dendrites on Cortical Pyramidal Neurons in Neuronal Storage Diseases Correlates with Abnormal Accumulation of GM2 Ganglioside

1994 • 86 citations

Selective reentry of recycling cell surface glycoproteins to the biosynthetic pathway in human hepatocarcinoma HepG2 cells.

1995 • 59 citations

Receptor extracellular domains may contain trafficking information. Studies of the 300-kDa mannose 6-phosphate receptor.

1994 • 50 citations

Degradation and regurgitation of extracellular proteins by cultured mouse peritoneal macrophages and baby hamster kidney fibroblasts. Kinetic evidence that the transfer of proteins to lysosomes is not irreversible.

1987 • 44 citations

Related membrane domains in proteins of sterol sensing and cell signaling provide a glimpse of treasures still buried within the dynamic realm of intracellular metabolic regulation

1998 • 44 citations

Balancing Needs, Efficiency, and Functionality in the Provision of Modeling Software: A Perspective of the NIH WinSAAM Project

1998 • 43 citations

Comparative behavior of lysosomes and the pre-lysosome compartment (PLC) in in vivo cell fusion experiments

1991 • 35 citations

Cycling of Two Endogenous Lysosomal Membrane Proteins, Lamp-2 and Acid Phosphatase, between the Cell Surface and Lysosomes in Cultured Rat Hepatocytes

1993 • 33 citations

A multicompartmental model of fluid-phase endocytosis in rabbit liver parenchymal cells

1989 • 31 citations

Accumulation of GM2 Ganglioside in Niemann-Pick Disease Type C Fibroblasts.

1996 • 27 citations

Elevated GM2 ganglioside is associated with dendritic proliferation in normal developing neocortex

1996 • 26 citations

[14C]methylamine accumulation in cultured human skin fibroblasts — a biochemical test for lysosomal storage and lysosomal diseases

1994 • 16 citations

Drastically abnormal gluco- and galactosylceramide composition does not affect ganglioside metabolism in the brain of mice deficient in galactosylceramide synthase.

1999 • 12 citations

A Chinese hamster ovary cell line with a temperature-conditional defect in receptor recycling is pleiotropically defective in lysosome biogenesis.

1993 • 10 citations

Methylamine accumulation in cultured cells as a measure of the aqueous storage compartment in the laboratory diagnosis of genetic lysosomal diseases

1996 • 8 citations