This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.
Immunogen Information
Immunogen
Recombinant fusion protein of human HIBCH
Gene ID
26275
Swissprot
Q6NVY1
Synonyms
HIBCHHIBYLCOAH
Observed MW
104kDa
Applications
Reactivity
Human,Mouse,Rat
Tested Applications
WB,IF
Conjugation
Unconjugated
Dilution
WB 1:500-1:2000,IF 1:50-1:100
Concentration
1mg/mL
Storage Buffer
PBS with 0.01% thiomersal,50% glycerol,pH7.3.
Storage Instructions
Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.