Article
[Proteasome degradation of protein C and plasmin inhibitor mutants: molecular mechanism of congenital protein deficiency].
Rinsho byori. The Japanese journal of clinical pathology - 1 Aug 2009
Nishio Miwako, Koyama Takatoshi, Hirosawa Shinsaku
Abstract excerpt
In many inherited disorders, protein deficiency is one of the major aetiologies, but the molecular and cellular mechanisms remain unclear. We investigated the intracellular degradation of mutant proteins, using naturally occurring PC and PI mutants that lead to congenital deficiencies. We have shown that proteasomes are very important for the degradation of PC and PI mutants, irrespective of the presence or...
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