General description
Translocase of inner mitochondrial membrane 8A (TIMM8A) is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms. TIMM8A gene is located on human chromosome Xq22. It is a 11 kDa protein. It belongs to a family of evolutionary conserved proteins, which are arranged in hetero-oligomeric complexes in the mitochondrial intermembrane space.
Immunogen
TIMM8A (NP_004076, 9 a.a. ~ 97 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
AAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
Application
Monoclonal Anti-TIMM8A antibody produced in mouse has been used in immunoblotting.
Biochem/physiol Actions
Translocase of inner mitochondrial membrane 8A (TIMM8A) is involved in the dynamin-1-like protein (Drp1)-mediated mitochondrial fission during programmed cell death. It interacts with TIMM13 and aids the import of TIMM23 into mitochondria.{152)
Physical form
Solution in phosphate buffered saline, pH 7.4
Legal Information
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- UPC:
- 51263173
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- No
- MPN:
- WH0001678M1-100UG
- Temperature Control Device:
- Yes
