Anti-HIBADH antibody (bs-15483R)

Anti-HIBADH antibody

DATASHEET

Host:Rabbit

Target Protein:HIBADH

IR:Immunogen Range:251-336/336

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:11112

Swiss Prot:P31937

Source:KLH conjugated synthetic peptide derived from human HIBADH:251-336/336 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Background:HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-HydroJNCisobutyrate 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-HydroJNCisobutyric 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-HydroJNCisobutyric aciduria as compared with controls.

Size:100ul

Concentration:1mg/ml

Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)

Cross Reactive Species:Human
Mouse
Rat
Cow
Horse
Sheep
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Sample:
193T(Human) Cell Lysate at 30 ug
MCF-7(Human) Cell Lysate at 30 ug
HepG2(Human) Cell Lysate at 30 ug
Kidney (Mouse) Lysate at 40 ug
Primary: Anti- HIBADH (bs-15483R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 32 kD
Observed band size: 30 kD