鸟氨酸氨基甲酰转移酶抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 鸟氨酸氨基甲酰转移酶抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous鸟氨酸氨基甲酰转移酶抗体 females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

鸟氨酸氨基甲酰转移酶抗体Also known as:

OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine Carbamoyltransferase; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.

Specificity:

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Rabbit Polyclonal IgG,鸟氨酸氨基甲酰转移酶抗体 affinity purified by Protein A.

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

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Immunogen: KLH conjugated synthetic peptide derived from human OTC (33-80aa).

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Predicted Molecular Weight: 40kDa.

鸟氨酸氨基甲酰转移酶抗体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  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user

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