3-羟基异丁酸脱氢酶抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 3-羟基异丁酸脱氢酶抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal 3-羟基异丁酸脱氢酶抗体activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.

3-羟基异丁酸脱氢酶抗体Also known as:

3 hydroxy 2 methylpropanoate:NAD(+) oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.

Specificity:

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Rabbit Polyclonal IgG,3-羟基异丁酸脱氢酶抗体 affinity purified by Protein A.

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

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Immunogen: KLH conjugated synthetic peptide derived from human HIBADH.

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Predicted Molecular Weight: 32kDa.

3-羟基异丁酸脱氢酶抗体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  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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