Abstract:
:Biogenesis of respiratory chain complexes depends on the expression of mitochondrial-encoded subunits. Their synthesis occurs on membrane-associated ribosomes and is probably coupled to their membrane insertion. Defects in expression of mitochondrial translation products are among the major causes of mitochondrial disorders. Mdm38 is related to Letm1, a protein affected in Wolf-Hirschhorn syndrome patients. Like Mba1 and Oxa1, Mdm38 is an inner membrane protein that interacts with ribosomes and is involved in respiratory chain biogenesis. We find that simultaneous loss of Mba1 and Mdm38 causes severe synthetic defects in the biogenesis of cytochrome reductase and cytochrome oxidase. These defects are not due to a compromised membrane binding of ribosomes but the consequence of a mis-regulation in the synthesis of Cox1 and cytochrome b. Cox1 expression is restored by replacing Cox1-specific regulatory regions in the mRNA. We conclude, that Mdm38 and Mba1 exhibit overlapping regulatory functions in translation of selected mitochondrial mRNAs.
journal_name
Mol Biol Celljournal_title
Molecular biology of the cellauthors
Bauerschmitt H,Mick DU,Deckers M,Vollmer C,Funes S,Kehrein K,Ott M,Rehling P,Herrmann JMdoi
10.1091/mbc.e10-02-0101subject
Has Abstractpub_date
2010-06-15 00:00:00pages
1937-44issue
12eissn
1059-1524issn
1939-4586pii
E10-02-0101journal_volume
21pub_type
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