Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
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- Product number
- BS61107 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- HIBADH polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- A synthetic peptide corresponding to residues in Human HIBADH.
- Description
- 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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
- Main image
- Experimental details
- Western blot (WB) analysis of HIBADH pAb at 1:500 dilution Lane1:Lane2:RAW264.7 whole cell lysate Lane3:H9C2 whole cell lysate Lane4:HELA whole cell lysate