(Q37118167)

English

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

scientific article published on February 2000

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scholarly article

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency (English)

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

molecular chaperones

0 references

1 reference

based on heuristic

inferred from title

pulmonary emphysema

1 reference

based on heuristic

inferred from title

author name string

J A Burrows

1

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

L K Willis

2

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

D H Perlmutter

3

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

language of work or name

0 references

publication date

1 February 2000

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

Proceedings of the National Academy of Sciences of the United States of America

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

97

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

4

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

1796-1801

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

Defective aquaporin-2 trafficking in nephrogenic diabetes insipidus and correction by chemical chaperones

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

Conformational disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

Correcting temperature-sensitive protein folding defects

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

Chemical chaperones interfere with the formation of scrapie prion protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

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.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

14 September 2017

Quantum proteolysis by neutrophils: implications for pulmonary emphysema in alpha 1-antitrypsin deficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

29 September 2017

Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

29 September 2017

Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

27 June 2018

Prospective treatment of urea cycle disorders.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

27 June 2018

Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26515

3 September 2018

The alpha 1-antitrypsin gene is expressed in a human intestinal epithelial cell line

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The effect of the Z mutation on the ability of alpha 1-antitrypsin to prevent neutrophil mediated tissue damage

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The liver in adolescents with α-antitrypsin deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structural and functional characterization of the abnormal Z alpha 1-antitrypsin isolated from human liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/10677536

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1073/PNAS.97.4.1796

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

2000PNAS...97.1796B

0 references

release_kjgyqtuwhbbmdjv7iequcrm45m

1 reference

https://api.fatcat.wiki/v0/release/kjgyqtuwhbbmdjv7iequcrm45m

24 November 2022

based on heuristic

mapped directly with Wikidata item

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

18 August 2017

ResearchGate publication ID

0 references

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