{"product_id":"abcam-ab265888","title":"Abcam, ab265888, Human HIBADH knockout HeLa cell line","description":"\u003cp\u003eSize: 2 x 1000000Cells \/ vial \/ 1000000Cells \/ vial\u003cbr\u003e\nHIBADH KO cell line available to order. KO validated by Western blot. Free of charge wild type control available. Knockout achieved by using CRISPR\/Cas9, 298 bp insertion in exon 1 and Insertion of the selection cassette in exon 1. To order both knockout and wild-type control cells: select 2 x 1000000Cells\/vial. To order only knockout cells: select 1000000Cells\/vial.\u003cbr\u003e\nKey facts\u003cbr\u003e\nCell type:HeLa,\u003cbr\u003e\nSpecies or organism:Human,\u003cbr\u003e\nTissue:Cervix,\u003cbr\u003e\nForm:LiquidSee storage information,\u003cbr\u003e\nKnockout validation:Sanger Sequencing,Western blot,\u003cbr\u003e\nMutation description:Knockout achieved by using CRISPR\/Cas9, 298 bp insertion in exon 1 and Insertion of the selection cassette in exon 1,\u003cbr\u003e\nDisease:Adenocarcinoma\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nWe will provide viable cells that proliferate on revival.\u003cbr\u003e\nThis 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\u003cbr\u003e\nlimited use license\u003cbr\u003e\npatent pages\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nGene 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\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nHIBADH 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.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThis 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.\u003cbr\u003e\nPathways\u003cbr\u003e\nHIBADH 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.\u003cbr\u003e\nHIBADH 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.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845067755689,"sku":"ab265888","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab265888","provider":"Iright","version":"1.0","type":"link"}