Target intelligence / Profile preview

Mitochondrial import inner membrane translocase subunit Tim8 B (TIMM8B)

Target
TIMM8B
Molecular classification
Chaperone protein, Protein transporter, Mitochondrial import machinery, Other
01

Overview

Mitochondrial import inner membrane translocase subunit Tim8 B (TIMM8B) is a nuclear-encoded mitochondrial intermembrane space chaperone protein that is highly conserved and shares similarity with yeast Tim proteins[1][4][7]. TIMM8B forms complexes to facilitate the import and insertion of multi-pass transmembrane proteins and participate in protein trafficking across the mitochondrial inner membrane before their integration into the membrane[1][2][4]. It also functions as an auxiliary assembly factor important for the formation and stability of cytochrome c oxidase (Complex IV) in the mitochondrial respiratory chain[2]. Disease associations include Mohr-Tranebjaerg Syndrome and dystonia, as well as possible links to hereditary paraganglioma due to proximity to the SDHD gene[1][7]. There is currently no evidence that TIMM8B is a direct therapeutic drug target, nor are there approved drugs known to interact with this protein. Its function is primarily as a component of the mitochondrial import machinery, crucial for mitochondrial protein biogenesis and respiratory chain function[2][4][9].

Other names
DDP2DDPLFLJ21744MGC102866MGC117373DDP-like proteinDeafness dystonia protein 2Tim8 B
02

Biological functions

Protein trafficking into mitochondriaAssembly of respiratory chain Complex IVChaperone activity for multi-pass transmembrane proteins
03

Disease associations

Neurodegenerative disease (associated with Mohr-Tranebjaerg Syndrome, dystonia)Other
04

Safety considerations

Genetic deficiency can contribute to mitochondrial dysfunction and related syndromes

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