TIMM8A Antibody
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.
Product Specifications
CAS Number
9007-83-4
Specifications
UniProt
O60220
Host
Rabbit
Reactivity
Human, Mouse, Rat, Monkey
Immunogen
A human recombinant partial protein (amino acids A14-D97) was used as the immunogen for the TIMM8A antibody.
Clonality
Polyclonal
Isotype
IgG
Applications
WB, IHC-P, IF, FACS, Direct ELISA
Purity
Affinity purified
Format
Antigen affinity purified
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Reconstitution
Limitations
This TIMM8A antibody is available for research use only.
Storage Conditions
Formulation
0.5 mg/mL if reconstituted with 0.2ml sterile DI water
Applications Notes
Optimal dilution of the TIMM8A antibody should be determined by the researcher.
Location
Cytoplasmic
Image Legend
IHC staining of FFPE human testicular cancer with TIMM8A antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.
Documents
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