Abstract：
:Prohibitins form large protein and lipid scaffolds in the inner membrane of mitochondria that are required for mitochondrial morphogenesis, neuronal survival, and normal lifespan. Here, we have defined the interactome of PHB2 in mitochondria and identified DNAJC19, mutated in dilated cardiomyopathy with ataxia, as binding partner of PHB complexes. We observed impaired cell growth, defective cristae morphogenesis, and similar transcriptional responses in the absence of either DNAJC19 or PHB2. The loss of PHB/DNAJC19 complexes affects cardiolipin acylation and leads to the accumulation of cardiolipin species with altered acyl chains. Similar defects occur in cells lacking the transacylase tafazzin, which is mutated in Barth syndrome. Our experiments suggest that PHB/DNAJC19 membrane domains regulate cardiolipin remodeling by tafazzin and explain similar clinical symptoms in two inherited cardiomyopathies by an impaired cardiolipin metabolism in mitochondrial membranes.
journal_name
Cell Metabjournal_title
Cell metabolismauthors
Richter-Dennerlein R,Korwitz A,Haag M,Tatsuta T,Dargazanli S,Baker M,Decker T,Lamkemeyer T,Rugarli EI,Langer Tdoi
10.1016/j.cmet.2014.04.016subject
Has Abstractpub_date
2014-07-01 00:00:00pages
158-71issue
1eissn
1550-4131issn
1932-7420pii
S1550-4131(14)00182-Xjournal_volume
20pub_type
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