Ribosome-binding proteins Mdm38 and Mba1 display overlapping functions for regulation of mitochondrial translation.

[wolf-hirschhorn syndrome]

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 . Mdm 38 is related to Letm 1 , a protein affected in Wolf-Hirschhorn syndrome patients . Like Mba 1 and Oxa 1 , Mdm 38 is an inner membrane protein that interacts with ribosomes and is involved in respiratory chain biogenesis . We find that simultaneous loss of Mba 1 and Mdm 38 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 Cox 1 and cytochrome b . Cox 1 expression is restored by replacing Cox 1 -specific regulatory regions in the mRNA . We conclude , that Mdm 38 and Mba 1 exhibit overlapping regulatory functions in translation of selected mitochondrial mRNAs .

Diseases
Validation

Diseases presenting "respiratory chain complexes" symptom

classical phenylketonuria

inclusion body myositis

pyruvate dehydrogenase deficiency

wolf-hirschhorn syndrome

zellweger syndrome

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