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Anti-TIMM8A antibody (bs-11769R)

Anti-TIMM8A antibody (bs-11769R)

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Anti-TIMM8A antibody

英文名称TIMM8A
中文名称线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体
别    名DDP 1; DDP; DDP1; Deafness dystonia protein 1; Deafness/dystonia peptide; DFN 1; DFN1; MGC12262; Mitochondrial import inner membrane translocase subunit Tim8 A; MTS; TIM 8A; TIM8A; TIMM 8A; Translocase of inner mitochondrial membrane 8 homolog A; X linked deafness dystonia protein; TIM8A_HUMAN. 

DATASHEET

Host:Rabbit

Target Protein:TIMM8A

IR:Immunogen Range:31-97/97

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:1678

Swiss Prot:O60220

Source:KLH conjugated synthetic peptide derived from human TIMM8A:31-97/97 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Background:The majority of mitochondrial-directed proteins are encoded by the nuclear genome and are transported to the mitochondria via regulated processes involving the mitochondrial Tom and Tim proteins (1). The mitochondrial Tim protein family is comprised of a large group of evolutionarily conserved proteins that are found in most eukaryotes (1,2). Import of nuclear-encoded precursor proteins into and across the mitochondrial inner membrane is mediated by two distinct complexes, the Tim23 complex and the Tim22 complex, which differ in their substrate specificity (1). Defects in Tim proteins are implicated in several neuro-degenerative diseases, suggesting important roles for Tim proteins in development and health (3,4). Tim8A and Tim8B, which map to human chromosomes Xq22.1 and 11q23.1-q23.2, respectively, are conserved proteins of the mitochondrial intermembrane space, which are organized in hetero-oligomeric complex with Tim13 (5,6,7). Tim8A is highly expressed in fetal and adult brain (5). Tim8A is mutated in deafness dystonia syndrome, a novel type of disease that causes severe neurological defects, thought to be caused by a defective mitochondrial protein transport system (5,8).

Size:100ul

Concentration:1mg/ml

Applications:ELISA(1:5000-10000)

Cross Reactive Species:Human
Mouse
Rat
Pig
Cow
Sheep
.

For research use only. Not intended for diagnostic or therapeutic use.

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Anti-TIMM8A antibody (bs-11769...

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