Background:

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]

Specificity:

translocase of inner mitochondrial membrane 8 homolog A (yeast)

Alternative names:

DDP; DDP1; DFN1; MTS; TIM8

Species reactivity:

Human, Mouse, Rat

Host:

Mouse

Ig type:

PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.

Immunogen:

Full length human recombinant protein of human TIMM8A (NP_004076) produced in E.coli.

Purification & Purity:

Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)

Molecular weight:

5.3 kDa(Predicted)

Applications:

IHC, WB

Usage:

WB 1:500~2000, IHC 1:500

Storage:

PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.

Precautions:

For research use only, not for use in diagnostic procedure.

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