{"product_id":"abcam-ab266484","title":"Abcam, ab266484, Human ACADVL (VLCAD) knockout HEK-293T cell line","description":"\u003cp\u003eSize: 2 x 1000000Cells \/ vial \/ 1000000Cells \/ vial\u003cbr\u003e\nACADVL KO cell line available to order. KO validated by Western blot. Free of charge wild type control available. Knockout achieved by using CRISPR\/Cas9, Homozygous: 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:HEK-293T,\u003cbr\u003e\nSpecies or organism:Human,\u003cbr\u003e\nTissue:Kidney,\u003cbr\u003e\nForm:LiquidSee storage information,\u003cbr\u003e\nKnockout validation:Sanger Sequencing,Western blot,\u003cbr\u003e\nMutation description:Knockout achieved by using CRISPR\/Cas9, Homozygous: Insertion of the selection cassette in exon 1\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-ACADVL, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Zygosity-Homozygous, 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\nACADVL also known as VLCAD (Very Long-Chain Acyl-CoA Dehydrogenase) is an enzyme that plays an important role in the breakdown of long-chain fatty acids within the mitochondria. It has a molecular mass of approximately 70 kDa. VLCAD is expressed in various tissues with high levels found in the heart skeletal muscle and liver. These tissues are heavily reliant on fatty acid oxidation for energy production especially when glucose availability is low.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nACADVL functions within the mitochondrial matrix where it catalyzes the initial step of beta-oxidation by desaturating long-chain acyl-CoA molecules. This enzyme is a part of a larger complex that includes other mitochondrial dehydrogenases contributing to the degradation of fatty acids into acetyl-CoA units. The process is essential for energy production especially under fasting conditions or high-energy demand scenarios such as muscle contraction and cardiac function.\u003cbr\u003e\nPathways\u003cbr\u003e\nVLCAD plays an essential role in the fatty acid beta-oxidation pathway. It works alongside other enzymes such as ACADM (Medium-Chain Acyl-CoA Dehydrogenase) to facilitate the sequential shortening of fatty acids which integrates into the Krebs cycle for further energy extraction. This pathway maximizes energy yield from fatty acids significantly influencing cellular metabolism and energy homeostasis.\u003cbr\u003e\nVLCAD deficiencies lead to severe metabolic conditions such as VLCAD deficiency. This disorder results in the impaired breakdown of long-chain fatty acids causing energy deficiency and the accumulation of toxic substances. Symptoms range from muscle weakness to serious cardiac complications. VLCAD deficiency's impact is often assessed in relation to proteins like ACSL1 and CPT2 which are involved in the fatty acid metabolism pathway and are central to effective energy processing in cells.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845078634665,"sku":"ab266484","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab266484","provider":"Iright","version":"1.0","type":"link"}