(Q40850434)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

title

Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

0 references

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

author name string

R Mahadeva

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

W S Chang

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

D J Oakley

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

R C Foreman

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

J Calvin

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

D G Wight

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

D A Lomas

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

language of work or name

English

0 references

publication date

1 April 1999

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

Journal of Clinical Investigation

4 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

volume

103

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

issue

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

page(s)

999-1006

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation.

4 December 2019

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Serpin alpha 1proteinase inhibitor probed by intrinsic tryptophan fluorescence spectroscopy

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Probing serpin reactive-loop conformations by proteolytic cleavage

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

A hinge region mutation in C1-inhibitor (Ala436-->Thr) results in nonsubstrate-like behavior and in polymerization of the molecule.

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

The serpin superfamily of proteinase inhibitors: structure, function, and regulation

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Biological implications of a 3 A structure of dimeric antithrombin

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp)

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

What do dysfunctional serpins tell us about molecular mobility and disease?

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Structure and variation of human alpha 1-antitrypsin

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Molecular basis of alpha-1-antitrypsin deficiency

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Hormone binding globulins undergo serpin conformational change in inflammation

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Implications of the three-dimensional structure of alpha 1-antitrypsin for structure and function of serpins

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

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

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Cirrhosis and heterozygous alpha1-antitrypsin deficiency in a 4-year-old girl

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Heterozygous alpha 1-antitrypsin deficiency and cirrhosis in adults, a fortuitous association

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

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

24 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Alpha 1-antitrypsin deficiency: evaluation of bronchiectasis with CT.

25 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Fluorescence resonance energy transfer

17 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Antithrombin-TRI (Ala382 to Thr) causing severe thromboembolic tendency undergoes the S-to-R transition and is associated with a plasma-inactive high-molecular-weight complex of aggregated antithrombin

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

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

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Occurrence of Alpha-1-antitrypsin Deficiency in 155 Patients with Alcoholic Liver Disease

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Studies in alpha 1-antitrypsin deficiency.

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=408255

Study of familial alpha-1-proteinase inhibitor deficiency including a rare proteinase inhibitor phenotype (IZ). I. Alpha-1-phenotyping and clinical investigations

19 August 2018

1 reference

12 December 2020

Structural explanation for the deficiency of S alpha 1-antitrypsin

1 reference

12 December 2020

Serum Pi types in some European, American, Asian and African populations

1 reference

12 December 2020

Cirrhosis Associated with Partial Deficiency of Alpha-1 Antitrypsin in an Adult

1 reference

12 December 2020

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

1 reference

12 December 2020

Structural evidence for methionine at the reactive site of human alpha-1-proteinase inhibitor

1 reference

12 December 2020

Heterozygous MZ alpha 1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Molecular characterisation of three alpha-1-antitrypsin deficiency variants: proteinase inhibitor (Pi) nullcardiff (Asp256----Val); PiMmalton (Phe51----deletion) and PiI (Arg39----Cys)

1 reference

12 December 2020

Siiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new alpha 1-antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone

1 reference

12 December 2020

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

1 reference

12 December 2020

Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin

1 reference

12 December 2020

Variants of alpha 1-antitrypsin in Puerto Rican children with asthma

1 reference

12 December 2020

Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis

1 reference

12 December 2020

Alpha 1-antitrypsin Siiyama (Ser53-->Phe). Further evidence for intracellular loop-sheet polymerization

1 reference

12 December 2020

Preparation and characterization of latent alpha 1-antitrypsin

1 reference

12 December 2020

The PiZ gene of alpha 1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis

1 reference

12 December 2020

Identifiers

DOI

10.1172/JCI4874

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10194472%20AND%20SRC:MED&resulttype=core&format=json

4 December 2019

PubMed publication ID

10194472

1 reference