Display Settings:

Format

Send to:

Choose Destination
Thorax. 2004 Jun;59(6):529-35.

Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology.

Author information

  • 1Respiratory Medicine Unit, Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Cambridge CB2 2XY, UK. dal16@cam.ac.uk

Abstract

The molecular basis of alpha(1)-antitrypsin deficiency is reviewed and is shown to be due to the accumulation of mutant protein as ordered polymers within the endoplasmic reticulum of hepatocytes. The current goals are to determine the cellular response to polymeric alpha(1)-antitrypsin and to develop therapeutic strategies to block polymerisation in vivo.

PMID:
15170041
[PubMed - indexed for MEDLINE]
PMCID:
PMC1747018
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for HighWire Icon for PubMed Central
    Loading ...
    Write to the Help Desk