YRP15488-01
[Polyclonal Antibody]
HIBADH Rabbit Polyclonal Antibody
www.yhsbio.com
market@yhsbio.com
support@yhsbio.com
+86-21-54651191
Room 703,Building 6,333# Guiping
Rd.,Xuhui District,Shanghai,China
market@yhsbio.com
support@yhsbio.com
+86-21-54651191
Room 703,Building 6,333# Guiping
Rd.,Xuhui District,Shanghai,China
DATASHEET
| Species: | Rabbit |
| Applications: | WB IHC IF |
| Immunogen Range: | KLH conjugated synthetic peptide derived from human HIBADH |
| Clonality: | Polyclonal Antibody |
| Isotype: | IgG |
| GENE ID: | 11112 |
| Swiss Prot: | |
| Synonyms: | 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase, 3 hydroxyisobutyrate dehydrogenase, 3 hydroxyisobutyrate dehydrogenase mitochondrial, EC 1.1.1.31, MGC40361, NS5ATP1, 3HIDH_HUMAN. |
| Purification: | Purified by Protein A. |
| Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
| 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 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. |
| Caculated MW: | / |
| Observed MW: | Refer to Figures |
| Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
| Reacitivity: | Human |
For research use only. Not intended for diagnostic or therapeutic use!
Additional information