线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体

Quantity size:

0.2ml

Concentration:

1mg/ml  线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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, 线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体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).线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体 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).

Also known as:

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;线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体 Translocase of inner mitochondrial membrane 8 homolog A; X linked deafness dystonia protein; TIM8A_HUMAN.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Pig, Cow, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human TIMM8A (31-97aa).

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线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体Predicted Molecular Weight: 11kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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