Anti-HIBADH Rabbit Polyclonal Antibody

Lieferant: Bioss
BS-15483R
BOSSBS-15483REA 725 EUR
BOSSBS-15483R
Anti-HIBADH Rabbit Polyclonal Antibody
Antikörper
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.

  • WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)

Type: Primary
Antigen: HIBADH
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human
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