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Article

Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer.

EMBO reports - 30 Sept 2011

Yamasaki Masayuki, Sendall Timothy J, Pearce Mary C, Whisstock James C, Huntington James A

Abstract excerpt

α(1)-Antitrypsin (α1AT) deficiency is a disease with multiple manifestations, including cirrhosis and emphysema, caused by the accumulation of stable polymers of mutant protein in the endoplasmic reticulum of hepatocytes. However, the molecular basis of misfolding and polymerization remain unknown. We produced and crystallized a trimeric form of α1AT that is recognized by an antibody specific for the pathological...

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Identifiers

PMID: 21909074
DOI: 10.1038/embor.2011.171
PMCID: PMC3185345

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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