Target intelligence / Profile preview

Mitochondrial import inner membrane translocase subunit Tim10 (TIMM10)

Target
TIMM10
Molecular classification
Transporter, Chaperone, Protein complex subunit
01

Overview

Mitochondrial import inner membrane translocase subunit Tim10 (TIMM10) is a small evolutionarily conserved protein that acts as a chaperone in the mitochondrial intermembrane space[3][4][5]. It is a core component of the TIM22 complex, which mediates the import and insertion of multi-pass transmembrane proteins, particularly metabolite carriers, into the inner mitochondrial membrane[1][3][4][5]. TIMM10 forms a heterooligomeric complex with other small Tim proteins (such as Tim9 and Tim12) and protects hydrophobic precursors from aggregation, ensuring their proper transfer and insertion. Dysfunction or mutation in TIMM10 impairs mitochondrial bioenergetics and can cause recessive disorders such as Mohr-Tranebjaerg syndrome, characterized by progressive neurodegeneration[3][5][7]. Key biological processes involving TIMM10 relate to mitochondrial integrity and metabolic competence, as it is indispensable for the assembly and maintenance of the mitochondrial inner membrane protein composition[1][3][5].

Other names
TIM10TIM10ATIMM10AMitochondrial import inner membrane translocase subunit Tim10 homologTranslocase of inner mitochondrial membrane 10Translocase of inner mitochondrial membrane 10 homolog (yeast)TIMM10 (yeast) homolog
02

Biological functions

Mitochondrial protein importProtein translocationProtein chaperoningInsertion of hydrophobic membrane proteins into mitochondrial inner membraneProtein complex assembly
03

Disease associations

Neurodegenerative disease (e.g., Mohr-Tranebjaerg syndrome)Other (rare mitochondrial disorders)
04

Safety considerations

Loss-of-function mutations in TIMM10 can cause Mohr-Tranebjaerg syndrome and other mitochondrial diseases, leading to neurodegeneration[3][7].Mutations affecting protein import can result in impaired mitochondrial function.

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