(Q41509050)

1 October 2017

1 reference

title

Review: alpha 1-antitrypsin deficiency associated liver disease (English)

1 reference

alpha-1-antitrypsin deficiency

1 October 2017

1 reference

Qu D

1

1 reference

PubMed ID

9195389

1 October 2017

Teckman JH

series ordinal

2

1 reference

PubMed ID

9195389

1 October 2017

Perlmutter DH

series ordinal

3

1 reference

1 October 2017

1 May 1997

1 reference

Journal of Gastroenterology and Hepatology

1 October 2017

1 reference

1 October 2017

12

1 reference

1 October 2017

5

1 reference

1 October 2017

404-416

1 reference

Alpha-1-antitrypsin deficiency. High prevalence in the St. Louis area determined by direct population screening

1 October 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Molecular abnormality of human alpha1-antitrypsin variant (Pi-ZZ) associated with plasma activity deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

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.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Disruption of the Lys-290--Glu-342 salt bridge in human alpha 1-antitrypsin does not prevent its synthesis and secretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

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.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

The effect of amino acid substitutions at position 342 on the secretion of human alpha 1-antitrypsin from Xenopus oocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Histopathology of α1-antitrypsin liver disease in a transgenic mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Hepatocarcinogenesis is the sequel to hepatitis in Z#2 alpha 1-antitrypsin transgenic mice: histopathological and DNA ploidy studies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Liver disease in alpha-1-antitrypsin deficiency: prognostic indicators

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

High prevalence of viral infection in adults with homozygous and heterozygous alpha 1-antitrypsin deficiency and chronic liver disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Alpha1 Antitrypsin Liver Disease Differential Diagnosis of PAS-Positive, Diastase-resistant Globules in Liver Cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Adenoviruses as gene-delivery vehicles

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

In vivo gene delivery and stable transduction of nondividing cells by a lentiviral vector

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Antisense-oligonucleotide therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

The mechanism of Z alpha 1-antitrypsin accumulation in the liver

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

The Z type variation of human alpha 1-antitrypsin causes a protein folding defect

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Protein sorting by transport vesicles

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

COPI- and COPII-coated vesicles bud directly from the endoplasmic reticulum in yeast.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

An integral membrane component of coatomer-coated transport vesicles defines a family of proteins involved in budding

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Protein folding in the cell

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Participation of a novel 88-kD protein in the biogenesis of murine class I histocompatibility molecules

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Association of folding intermediates of glycoproteins with calnexin during protein maturation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

The molecular chaperone calnexin binds Glc1Man9GlcNAc2 oligosaccharide as an initial step in recognizing unfolded glycoproteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Pre-Golgi degradation of newly synthesized T-cell antigen receptor chains: intrinsic sensitivity and the role of subunit assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Misfolded major histocompatibility complex class I molecules accumulate in an expanded ER-Golgi intermediate compartment

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

The cellular basis for liver injury in alpha 1-antitrypsin deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Regulation of MHC class I transport by the molecular chaperone, calnexin (p88, IP90).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Calnexin: a membrane-bound chaperone of the endoplasmic reticulum

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Gamma-interferon and expression of MHC genes regulate peptide hydrolysis by proteasomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Molecular pathogenesis of hepatocellular carcinoma in hepatitis B virus transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Hepatitis B virus transgenic mice: insights into the virus and the disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Amino acid control of autophagic sequestration and protein degradation in isolated rat hepatocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Transcriptional induction of genes encoding endoplasmic reticulum resident proteins requires a transmembrane protein kinase

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

A transmembrane protein with a cdc2+/CDC28-related kinase activity is required for signaling from the ER to the nucleus

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Activation of transcription factor NF-kappaB by the adenovirus E3/19K protein requires its ER retention

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Induced thermal resistance in HeLa cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

α1-Antitrypsin deficiency detection by direct analysis of the mutation in the gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Sequential interaction of the chaperones BiP and GRP94 with immunoglobulin chains in the endoplasmic reticulum

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

Mobile reactive centre of serpins and the control of thrombosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1440-1746.1997.TB00451.X

10.1111/J.1440-1746.1997.TB00451.X

21 January 2018

Identifiers

DOI

1 reference

1 October 2017

1 reference

PubMed ID

9195389