Glucosidase and Mannosidase Inhibitors Mediate Increased Secretion of Mutant α1 Antitrypsin Z

The Biogenesis of Lysosomes

1989 • 1,583 citations

The mechanism of Z α1-antitrypsin accumulation in the liver

1992 • 1,030 citations

Glucose trimming and reglucosylation determine glycoprotein association with calnexin in the endoplasmic reticulum

1995 • 544 citations

Mutations in the ER–Golgi Intermediate Compartment Protein ERGIC-53 Cause Combined Deficiency of Coagulation Factors V and VIII

1998 • 461 citations

Calnexin, calreticulin and the folding of glycoproteins

1997 • 377 citations

Degradation of a Mutant Secretory Protein, α1-Antitrypsin Z, in the Endoplasmic Reticulum Requires Proteasome Activity

1996 • 362 citations

Degradation of Misfolded Endoplasmic Reticulum Glycoproteins in Saccharomyces cerevisiae Is Determined by a Specific Oligosaccharide Structure

1998 • 344 citations

A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.

1994 • 293 citations

Glycosylation inhibitors in biology and medicine

1995 • 278 citations

Oligosaccharide Modification in the Early Secretory Pathway Directs the Selection of a Misfolded Glycoprotein for Degradation by the Proteasome

1999 • 235 citations

Novel Aspects of Degradation of T Cell Receptor Subunits from the Endoplasmic Reticulum (ER) in T Cells: Importance of Oligosaccharide Processing, Ubiquitination, and Proteasome-dependent Removal from ER Membranes

1998 • 218 citations

Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase.

1987 • 207 citations

Conformation-Independent Binding of Monoglucosylated Ribonuclease B to Calnexin

1997 • 195 citations

Quality Control in the Secretory Pathway: The Role of Calreticulin, Calnexin and BiP in the Retention of Glycoproteins with C-Terminal Truncations

1997 • 187 citations

Quantum proteolysis by neutrophils: implications for pulmonary emphysema in α1-antitrypsin deficiency

1999 • 136 citations

The Z type variation of human α1-antitrypsin causes a protein folding defect

1995 • 136 citations

Mannose-dependent Endoplasmic Reticulum (ER)-Golgi Intermediate Compartment-53-mediated ER to Golgi Trafficking of Coagulation Factors V and VIII

1999 • 134 citations

Intracellular Disposal of Incompletely Folded Human α1-Antitrypsin Involves Release from Calnexin and Post-translational Trimming of Asparagine-linked Oligosaccharides

1997 • 124 citations

Persistence of glucose residues on core oligosaccharides prevents association of TCR alpha and TCR beta proteins with calnexin and results specifically in accelerated degradation of nascent TCR alpha proteins within the endoplasmic reticulum.

1994 • 117 citations

Molecular pathogenesis of liver disease in ?1-antitrypsin deficiency

1996 • 110 citations

Inhibition of glucose trimming by castanospermine results in rapid degradation of unassembled major histocompatibility complex class I molecules.

1993 • 86 citations

Structural and functional characterization of the abnormal Z α₁‐antitrypsin isolated from human liver

1984 • 76 citations

Endoplasmic Reticulum Kifunensine-Resistant α-Mannosidase Is Enzymatically and Immunologically Related to the Cytosolic α-Mannosidase

1996 • 75 citations

Glycosylation of human α1-antitrypsin inSaccharomyces cerevisiae and methylotrophic yeasts

1998 • 61 citations

Effect of glycosylation on the stability of α1-antitrypsin toward urea denaturation and thermal deactivation

1997 • 59 citations

Different effects of the glucosidase inhibitors 1-deoxynojirimycin, N-methyl-1-deoxynojirimycin and castanospermine on the glycosylation of rat α1-proteinase inhibitor and α1-acid glycoprotein

1986 • 47 citations

Processing of viral envelope glycoprotein by the endomannosidase pathway: Evaluation of host cell specificity

1998 • 44 citations

Secretion and apparent activation of human hepatic lipase requires proper oligosaccharide processing in the endoplasmic reticulum

1998 • 24 citations

Castanospermine analogues: their inhibition of glycoprotein processing α-glucosidases from porcine kidney and B₁₆F₁₀ cells

1995 • 17 citations