GTX64883
TIMM8A antibody
Cannot supply to this region.
- SKU:
- GTX64883
- Additional Names:
- translocase of inner mitochondrial membrane 8A , DDP , DDP1 , DFN1 , MTS , TIM8
- Application:
- WB
- Physical State:
- Liquid
- Species Reactivity:
- Human
- Purification:
- Affinity Purified
- Storage Conditions:
- -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
- Supplier:
- Genetex
- Host:
- Rabbit
- Reactivities:
- Human, Mouse
- Buffer:
- PBS, 50% Glycerol, 0.02% Sodium azide.
- Immunogen:
- Recombinant fusion protein containing a sequence corresponding to amino acids 1-97 of human TIMM8A (NP_004076.1).
- Uniprot:
- O60220
- Synonyms:
- 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
- Extra Details:
- 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]
- Shipping Conditions:
- Blue Ice