共济失调性眼球运动功能丧失相关蛋白AOA1抗体

?共济失调性眼球运动功能丧失相关蛋白AOA1抗体Quantity size:

0.2ml

Concentration:

1mg/ml   Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

Aprataxin is a nuclear protein, present in both the nucleoplasm and the nucleolus, which is a member of the histidine triad (HIT) superfamily. Aprataxin is involved in DNA single-strand break repair, mediating protein-protein interactions with molecules responding to DNA damage. 共济失调性眼球运动功能丧失相关蛋白AOA1抗体Aprataxin contains three conserved domains: an N-terminal forkhead-associated (FHA) domain which mediates protein-protein interactions, a HIT domain that is similar to Hint, and a C-terminal zinc finger domain. Loss of function mutations in APTX, the gene encoding for Aprataxin, destabilize the Aprataxin protein and result in a rare neurological disorder known as ataxia-oculomotor apraxia, characterized by abnormal movements of the head and eyes. These mutations either target the HIT domain or truncate the protein N-terminal to a zinc finger.

共济失调性眼球运动功能丧失相关蛋白AOA1抗体Also known as:

AOA 1; AOA; AOA; AOA1; AOA1; Aprataxin; Aprataxin; Aprataxin homolog; APTX; APTX; APTX_HUMAN; Ataxia 1 early onset with hypoalbuminemia; Ataxia 1 early onset with hypoalbuminemia; Ataxia1 early onset with hypoalbuminemia; AXA 1; AXA1; AXA1; EAOH; EAOH; EOAHA; EOAHA; FHA HIT; FHA HIT; FHA-HIT; FLJ20157; FLJ20157; Forkhead associated domain histidine triad like; Forkhead associated domain histidine triad like; Forkhead associated domain histidine triad like protein;共济失调性眼球运动功能丧失相关蛋白AOA1抗体 Forkhead-associated domain histidine triad-like protein; MGC1072; MGC1072.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Horse, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human Aprataxin.

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共济失调性眼球运动功能丧失相关蛋白AOA1抗体Predicted Molecular Weight: 41kDa.

Storage:

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

Application:

WB=1:100-500  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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