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Mitochondrial import inner membrane translocase subunit Tim13 (TIMM13)
- Target
- TIMM13
- Molecular classification
- Other (mitochondrial import machinery), Chaperone protein
01
Overview
Mitochondrial import inner membrane translocase subunit Tim13 (TIMM13) is a small chaperone protein located in the mitochondrial intermembrane space. It forms a complex with TIMM8A, mediating the import and assembly of multi-pass transmembrane proteins, including key components such as TIMM23 and certain solute carrier family members, into the inner mitochondrial membrane. TIMM13 protects precursor proteins from aggregation in the intermembrane space, ensuring their correct targeting and insertion. It is essential for overall mitochondrial integrity and function, with roles in mitochondrial protein import, metabolism, and maintenance of cellular homeostasis. Upregulation of TIMM13 has been linked to tumor cell proliferation and migration in osteosarcoma, making it a proposed novel therapeutic target; defects in TIMM13 or its complex contribute to neurodegenerative diseases such as Mohr-Tranebjaerg syndrome[2][3][6].
- Other names
- TIM13TIMM13ATIM13BTIMM13BTim13PPV1mitochondrial import inner membrane translocase subunit Tim13Btranslocase of inner mitochondrial membrane 13 homolog
02
Biological functions
Protein import into mitochondriaChaperone activityMitochondrial integrityProtein insertion into mitochondrial inner membrane
03
Disease associations
Neurodegenerative disease (Mohr-Tranebjaerg syndrome)Cancer (notably osteosarcoma)Other (mitochondrial dysfunction disorders)
04
Safety considerations
Disruption can lead to mitochondrial dysfunction, increased oxidative stress, cell death; unknown as a therapeutic risk due to lack of specific drugs targeting TIMM13[2]
05
Biomarkers
Overexpression predicts poor prognosis in osteosarcoma[2]
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