Target intelligence / Profile preview

Translocase of inner mitochondrial membrane 10B (TIMM10B)

Target
TIMM10B
Molecular classification
Transporter (specifically, mitochondrial protein translocase subunit), Enzyme (some sources categorize the Tim proteins as enzymes due to chaperone and assembly functions), Other (chaperone, part of TIM22 complex)
01

Overview

Translocase of inner mitochondrial membrane 10B (TIMM10B) is an evolutionarily conserved protein found in the mitochondrial intermembrane space, functioning as a core component of the TIM22 complex. This complex is responsible for mediating the import and insertion of multi-pass transmembrane carrier proteins into the mitochondrial inner membrane, which is crucial for proper mitochondrial function. TIMM10B acts as part of a heterooligomeric chaperone assembly, facilitating the docking, translocation, and membrane integration of carrier protein precursors. Disruption or mutation of TIMM10B is associated with hereditary mitochondrial disorders such as Sengers syndrome and Mohr-Tranebjaerg syndrome, and recent literature links this protein to central nervous system diseases.

Other names
TIMM10BTIM10BTim9bFXC1Mitochondrial import inner membrane translocase subunit Tim10 BMitochondrial import inner membrane translocase subunit Tim9 BFracture Callus Protein 1Translocase of inner mitochondrial membrane 10 homolog B (yeast)
02

Biological functions

Protein import into the mitochondrial inner membraneInsertion of multi-pass transmembrane proteinsChaperoning hydrophobic protein precursors within the mitochondrial intermembrane space
03

Disease associations

Neurodegenerative disease (literature association with central nervous system disorders)Sengers syndromeMohr-Tranebjaerg syndrome

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