{"product_id":"abcam-ab266471","title":"Abcam, ab266471, Human CHCHD3 (MIC19) knockout HEK-293T cell line","description":"\u003cp\u003eSize: 2 x 1000000Cells \/ vial \/ 1000000Cells \/ vial\u003cbr\u003e\nCHCHD3 KO cell line available to order. KO validated by. Free of charge wild type control available. Knockout achieved by using CRISPR\/Cas9, Homozygous: 1 bp deletion 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,\u003cbr\u003e\nMutation description:Knockout achieved by using CRISPR\/Cas9, Homozygous: 1 bp deletion 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 and ERS Genomics Limited, and is developed with patented technology. For full details of the limited use licenses and relevant 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-CHCHD3, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, 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\nMIC19 also known as mitofilin is an integral component of the inner mitochondrial membrane part of the mitochondrial inner membrane organizing system (MINOS). It has a molecular mass of approximately 86 kDa. Expression of MIC19 occurs mainly in tissues with high energy demands including the heart and skeletal muscles. It plays a mechanical role in maintaining the structural integrity of mitochondrial cristae by anchoring specific protein components within the membrane.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nMIC19 contributes to the organization and functional maintenance of mitochondrial architecture by participating in the MINOS complex. This complex regulates the interaction between the inner and outer mitochondrial membranes ensuring efficient mitochondrial dynamics and biogenesis. Furthermore MIC19 supports mitochondrial bioenergetics by stabilizing cristae structures necessary for optimal electron transport chain function.\u003cbr\u003e\nPathways\u003cbr\u003e\nMIC19 integrates into the processes controlling mitochondrial fusion and fission dynamics. It functions within the pathways that are essential for cellular energy metabolism and apoptosis. MIC19 influences these pathways through interactions with proteins such as OPA1 which regulates inner membrane fusion and with components of the electron transport chain. This integrative role highlights MIC19's involvement in maintaining cellular energy homeostasis and programmed cell death.\u003cbr\u003e\nMIC19 associates with neurodegenerative diseases and cardiomyopathies. Altered expression or mutations in MIC19 can disrupt cristae architecture impairing mitochondrial function and energy production. This disruption relates to the disease processes seen in mitochondrial myopathy where proteins like OPA1 also play a role in mediating mitochondrial dysfunction. The interconnected roles of MIC19 and these associated proteins suggest potential therapeutic targets for intervention in mitochondrial-related conditions.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845206757545,"sku":"ab266471","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab266471","provider":"Iright","version":"1.0","type":"link"}