Dataset Information

Proteomic analysis of in situ formalin-fixed paraffin-embedded (FFPE) human liver tissues

ABSTRACT: in situ proteomic analysis (combining laser microdissection of hepatocytes and mass spectrometry analysis) on formalin-fixed paraffin-embedded (FFPE) human liver tissues of 4 ZZ-Alpha 1-Antitrypsin Deficiency (AATD) pediatric patients and 4 ZZ-AATD adult patients that underwent liver transplantation versus 3 WT individuals infants and 4 adults, and compared proteins differentially expressed in ZZ pediatric and in ZZ adult AATD patients.

INSTRUMENT(S): Orbitrap Fusion Lumos, Q Exactive

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Liver

DISEASE(S): Alpha 1-antitrypsin Deficiency

SUBMITTER: Dupuy Jean-William

LAB HEAD: Marion Bouchecareilh

PROVIDER: PXD022994 | Pride | 2021-09-09

REPOSITORIES: Pride

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Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency.

Karatas Esra E Raymond Anne-Aurélie AA Leon Céline C Dupuy Jean-William JW Di-Tommaso Sylvaine S Senant Nathalie N Collardeau-Frachon Sophie S Ruiz Mathias M Lachaux Alain A Saltel Frédéric F Bouchecareilh Marion M

JHEP reports : innovation in hepatology 20210424 4

<h4>Background & aims</h4>A single point mutation in the Z-variant of alpha 1-antitrypsin (Z-AAT) alone can lead to both a protein folding and trafficking defect, preventing its exit from the endoplasmic reticulum (ER), and the formation of aggregates that are retained as inclusions within the ER of hepatocytes. These defects result in a systemic AAT deficiency (AATD) that causes lung disease, whereas the ER-retained aggregates can induce severe liver injury in patients with ZZ-AATD. Unfortunate ...[more]

PMID: 34151245

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