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

Redesign of Retrovirus Packaging Cell Lines To Avoid Recombination Leading to Helper Virus Production

1986 • 1,276 citations

The mechanism of Z α1-antitrypsin accumulation in the liver

1992 • 1,030 citations

Liver Disease in Alpha₁-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

1976 • 787 citations

Expression of a foreign gene in myeloid and lymphoid cells derived from multipotent haematopoietic precursors

1985 • 598 citations

Progress in Liver Disease.

1976 • 572 citations

Inhibition of restriction endonuclease Nel I cleavage by phosphorothioate groups and its application to oligonucleotide-directed mutagenesis

1986 • 560 citations

Association of folding intermediates of glycoproteins with calnexin during protein maturation

1993 • 558 citations

Calnexin: a membrane-bound chaperone of the endoplasmic reticulum

1994 • 533 citations

Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy.

1990 • 500 citations

Degradation from the endoplasmic reticulum: Disposing of newly synthesized proteins

1988 • 488 citations

Hepatoma secretory proteins migrate from rough endoplasmic reticulum to Golgi at characteristic rates

1983 • 436 citations

Regulation of MHC Class I Transport by the Molecular Chaperone, Calnexin (p88, IP90)

1994 • 351 citations

Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.

1989 • 318 citations

Endoplasmic reticulum resident protein of 90 kilodaltons associates with the T- and B-cell antigen receptors and major histocompatibility complex antigens during their assembly.

1992 • 259 citations

The Natural History of Liver Disease in (α₁‐Antitrypsin Deficient Children

1988 • 242 citations

Neonatal Hepatitis Induced by α ₁ -Antitrypsin: a Transgenic Mouse Model

1988 • 139 citations

Cell-specific expression of alpha 1-antitrypsin in human intestinal epithelium.

1993 • 132 citations

Soluble aggregates of the human PiZ alpha 1-antitrypsin variant are degraded within the endoplasmic reticulum by a mechanism sensitive to inhibitors of protein synthesis.

1992 • 123 citations

Association between calnexin and a secretion-incompetent variant of human alpha 1-antitrypsin.

1994 • 112 citations

Intracellular degradation of the transport-impaired human PiZ alpha 1-antitrypsin variant. Biochemical mapping of the degradative event among compartments of the secretory pathway.

1990 • 111 citations

The cellular defect in alpha 1-proteinase inhibitor (alpha 1-PI) deficiency is expressed in human monocytes and in Xenopus oocytes injected with human liver mRNA.

1985 • 99 citations

Repair of the Secretion Defect in the Z Form of α1-Antitrypsin by Addition of a Second Mutation

1988 • 82 citations

Endoplasmic reticulum degradation of a subunit of the asialoglycoprotein receptor in vitro. Vesicular transport from endoplasmic reticulum is unnecessary.

1992 • 74 citations

Nonlysosomal, pre-Golgi degradation of unassembled asialoglycoprotein receptor subunits: a TLCK- and TPCK-sensitive cleavage within the ER.

1991 • 74 citations

Molecular Basis for Defective Secretion of the Z Variant of Human Alpha-1-Proteinase Inhibitor: Secretion of Variants Having Altered Potential for Salt Bridge Formation between Amino Acids 290 and 342

1989 • 72 citations

Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease.

1989 • 53 citations

Two pathways for the degradation of the H2 subunit of the asialoglycoprotein receptor in the endoplasmic reticulum.

1993 • 51 citations

Liver disease associated with alpha 1-antitrypsin deficiency.

1993 • 20 citations

[39] In situ detection of transcription in transfected cells using biotin-labeled molecular probes

1987 • 8 citations