δ氨基乙酰丙酸脱水酶抗体

?δ氨基乙酰丙酸脱水酶抗体Quantity size:

0.2ml

Concentration:

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

Background:

Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. Involvement in disease:Defects in ALAD are the cause of acute hepatic porphyria (AHP). δ氨基乙酰丙酸脱水酶抗体AHP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, δ氨基乙酰丙酸脱水酶抗体or endocrine factors.

Also known as:

ALAD; ALADH; ALADR; Aminolevulinate dehydratase; Aminolevulinate, delta, dehydratase; Delta aminolevulinic acid dehydratase; Delta-aminolevulinic acid dehydratase; HEM2_HUMAN; Lv; PBGS; Porphobilinogen synthase.

Specificity:

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Rabbit Polyclonal IgG,δ氨基乙酰丙酸脱水酶抗体 affinity purified by Protein A.

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

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Immunogen: KLH conjugated synthetic peptide derived from human ALAD.

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Predicted Molecular Weight: 36kDa.

δ氨基乙酰丙酸脱水酶抗体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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