Description
TIMM8A Antibody | RQ6157 | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: 0.5mg/ml if reconstituted with 0.2ml sterile DI water
Format: Antigen affinity purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human, Mouse, Rat, Monkey
Application: WB, IHC-P, IF, FACS, Direct ELISA
Buffer: N/A
Limitation: This TIMM8A antibody is available for research use only.
Purity: Affinity purified
Description: Mitochondrial import inner membrane translocase subunit Tim8 A, also known as Deafness-dystonia peptide or protein is an enzyme that in humans is encoded by the TIMM8A gene. 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.
Immunogen: A human recombinant partial protein (amino acids A14-D97) was used as the immunogen for the TIMM8A antibody.
Storage: After reconstitution, the TIMM8A antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20 °C. Avoid repeated freezing and thawing.
