Saturday, October 08, 2005

Mitochondrial Biology Gets A New Chaperone

Source: Journal of Clinical Investigation
Date Posted: 2005-10-07
Web Address: http://www.sciencedaily.com/releases/2005/10/051007101150.htm

MITOCHONDRIAL BIOLOGY GETS A NEW CHAPERONE

Mitochondrial complex I deficiency is one of the most common defects in
patients with mitochondrial disease. The deficiency results from a
failure to assemble the enzyme properly, but the nature of the
molecular chaperones that are necessary for this process in mammals
have remained obscure.

In a new study appearing on October 3 in The Journal of Clinical
Investigation, Eric Shoubridge and colleagues McGill University
identify candidate proteins involved in complex I assembly, and show
that one of the candidates, B17.2L, is an assembly factor. The authors identify a null mutation in a patient with a
progressive encephalopathy, and show that the defect can be
functionally complemented by expression of the wild-type cDNA in
patient cells. They also show that an antibody against the B17.2L
protein recognizes a subassembly of complex I in several additional
patients with complex I assembly defects, but not the whole enzyme
complex itself, consistent with a role as a molecular chaperone.

This is the first molecular chaperone to be characterized for mammalian
complex I, and is the first identification of the genetic basis of
disease in a patient with a complex I assembly defect.

In a related commentary, Robert Nussbaum writes, "The research
described here combines clever model organism genomics and
bioinformatics to identify the first mammalian protein required for the
normal assembly of complex I."

http://www.sciencedaily.com

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