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Titel:

Liver mitochondrial membrane crosslinking and destruction in a rat model of Wilson disease.

Dokumenttyp:
In Vitro; Journal Article; Research Support, Non-U.S. Gov't
Autor(en):
Zischka, H; Lichtmannegger, J; Schmitt, S; Jägemann, N; Schulz, S; Wartini, D; Jennen, L; Rust, C; Larochette, N; Galluzzi, L; Chajes, V; Bandow, N; Gilles, VS; DiSpirito, AA; Esposito, I; Goettlicher, M; Summer, KH; Kroemer, G
Abstract:
Wilson disease (WD) is a rare hereditary condition that is caused by a genetic defect in the copper-transporting ATPase ATP7B that results in hepatic copper accumulation and lethal liver failure. The present study focuses on the structural mitochondrial alterations that precede clinical symptoms in the livers of rats lacking Atp7b, an animal model for WD. Liver mitochondria from these Atp7b-/- rats contained enlarged cristae and widened intermembrane spaces, which coincided with a massive mitoch...     »
Zeitschriftentitel:
J Clin Invest
Jahr:
2011
Band / Volume:
121
Heft / Issue:
4
Seitenangaben Beitrag:
1508-18
Sprache:
eng
Volltext / DOI:
doi:10.1172/JCI45401
PubMed:
http://view.ncbi.nlm.nih.gov/pubmed/21364284
Print-ISSN:
0021-9738
TUM Einrichtung:
Institut für Allgemeine Pathologie und Pathologische Anatomie; Institut für Toxikologie und Umwelthygiene
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