鸟氨酸氨甲酰基线粒体抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 鸟氨酸氨甲酰基线粒体抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia.鸟氨酸氨甲酰基线粒体抗体 Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]

鸟氨酸氨甲酰基线粒体抗体Also known as:

EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.

Specificity:

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Rabbit Polyclonal IgG, 鸟氨酸氨甲酰基线粒体抗体affinity purified by Protein A.

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

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Immunogen: KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase.

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鸟氨酸氨甲酰基线粒体抗体Predicted Molecular Weight: 36kDa.

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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