Translocase Of Inner Mitochondrial Membrane 8B (TIMM8B)

TIMM8B encodes a member of a well-conserved family of proteins with similarity to yeast Tim mitochondrial import proteins. This gene is encoded by a nuclear gene and is transported into the intermembrane space of the mitochondrion. When formed into complexes, these proteins guide membrane-spanning proteins across the mitochondrial intermembrane space before they are added into the mitochondrial inner membrane. This gene is adjacent to succinate dehydrogenase, subunit D (SDHD), in which mutations have been found in affected members of families with hereditary paraganglioma.The 83-amino acid TIMM8B protein shares 45% sequence identity with TIMM8A. Northern blot analysis detected ubiquitous expression of 0.8-, 1.0-, and 3.5-kb TIMM8B transcripts in adult tissues, with highest expression in skeletal and cardiac muscle.