TIMM8A antibody

GeneTex

TIMM8A antibody

9

WB: 1:500 - 1:2000. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.

Western Blot

Research Use Only

Polyclonal

Unconjugated

TIMM8A

translocase of inner mitochondrial membrane 8A

DDP; DDP1; deafness dystonia protein 1; deafness/dystonia peptide; DFN1; mitochondrial import inner membrane translocase subunit Tim8 A; MTS; TIM8; translocase of inner mitochondrial membrane 8 homolog A; X-linked deafness dystonia protein

Rabbit

IgG

Mitochondrial import inner membrane translocase subunit Tim8 A

This translocase 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.[provided by RefSeq, Mar 2009]

Human, Mouse

-20°C or -80°C,2°C to 8°C

12352203