Description
Key facts
Cell type:HeLa,
Species or organism:Human,
Tissue:Cervix,
Form:LiquidSee storage information,
Knockout validation:Sanger Sequencing,Western blot,
Mutation description:Knockout achieved by using CRISPR/Cas9, 298 bp insertion in exon 1 and Insertion of the selection cassette in exon 1,
Disease:Adenocarcinoma
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-HIBADH, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
HIBADH known as 3-hydroxyisobutyrate dehydrogenase plays a role in the catabolic pathway of valine. It catalyzes the oxidation of 3-hydroxyisobutyrate to 3-oxopropanoate. This enzyme weighs approximately 33 kDa. Expression of HIBADH occurs predominantly in the liver kidney and to a lesser extent the heart and skeletal muscle tissues. High expression in these organs suggests its importance in energy metabolism and intermediate metabolite processing.
Biological function summary
This enzyme facilitates the metabolism of amino acids by converting valine-derived intermediates. HIBADH contributes significantly to energy production particularly during periods of fasting. It is not typically recognized as part of a larger protein complex functioning independently within metabolic pathways. Its role in energy homeostasis indicates its essential part in maintaining cellular function during metabolic stress.
Pathways
HIBADH is integral to the valine degradation pathway and also impacts the broader branched-chain amino acid catabolism process. These pathways are critical for recycling amino acids for ATP production. HIBADH interacts with other enzymes like acyl-CoA dehydrogenases which further process breakdown products. This interaction highlights HIBADH's connection to a consistent and efficient metabolic network ensuring effective energy yield from valine.
HIBADH has relevance to maple syrup urine disease and its metabolic management. Proper function of HIBADH is essential to avoid accumulation of toxic valine metabolites linked to this condition. Additionally connection to the protein BCKDHA hints at broader implications for amino acid metabolism disorders. BCKDHA part of the branched-chain alpha-ketoacid dehydrogenase complex indicates HIBADH's involvement in a critical intersection of metabolic control and disease prevention.
