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TAMM41

Function

Catalyzes the conversion of phosphatidic acid (PA) to CDP-diacylglycerol (CDP-DAG), an essential intermediate in the synthesis of phosphatidylglycerol, cardiolipin and phosphatidylinositol.

Involvement in disease

Combined oxidative phosphorylation deficiency 56

COXPD56

An autosomal recessive mitochondrial disease characterized by lethargy at birth, hypotonia, developmental delay, myopathy, and ptosis.

None

The disease may be caused by variants affecting the gene represented in this entry.

Pathway

Phospholipid metabolism; CDP-diacylglycerol biosynthesis; CDP-diacylglycerol from sn-glycerol 3-phosphate: step 3/3.

Sequence Similarities

Belongs to the TAM41 family.

Cellular localization

Alternative names

C3orf31, TAMM41, CDP-diacylglycerol synthase, Mitochondrial translocator assembly and maintenance protein 41 homolog, CDP-DAG synthase, TAM41

swissprot:Q96BW9 omim:614948 entrezGene:132001