HIBADH polyclonal, anti-human, mouse, rat
€305.00
In stock
SKU
BS61107
Background:
HIBADH (3-hydroxyisobutyrate dehydrogenase) 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 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.
Alternative Name:
3-hydroxyisobutyrate dehydrogenase, mitochondrial (EC:1.1.1.31), HIBADH
Application Dilution: WB: 1:500~1:1000
Specificity: HIBADH polyclonal antibody detects endogenous levels of HIBADH protein.
Immunogen:
A synthetic peptide corresponding to residues in Human HIBADH.
MW: ~ 35 kDa
Swis Prot.: P31937
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.3.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
HIBADH (3-hydroxyisobutyrate dehydrogenase) 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 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.
Alternative Name:
3-hydroxyisobutyrate dehydrogenase, mitochondrial (EC:1.1.1.31), HIBADH
Application Dilution: WB: 1:500~1:1000
Specificity: HIBADH polyclonal antibody detects endogenous levels of HIBADH protein.
Immunogen:
A synthetic peptide corresponding to residues in Human HIBADH.
MW: ~ 35 kDa
Swis Prot.: P31937
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.3.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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