{"product_id":"abcam-ab114664","title":"Abcam, ab114664, Recombinant Human Tafazzin \/ TAZ protein","description":"\u003cp\u003eSize: 10µg\u003cbr\u003e\nRecombinant Human Tafazzin \/ TAZ protein is a Human Full Length protein, in the 1 to 262 aa range, expressed in Wheat germ, suitable for SDS-PAGE, ELISA, WB.\u003cbr\u003e\nKey facts\u003cbr\u003e\nExpression system:Wheat germ,\u003cbr\u003e\nTags:GST tag N-Terminus,\u003cbr\u003e\nApplications:ELISA, SDS-PAGE, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nBiologically active:No,\u003cbr\u003e\nAccession:Q16635,\u003cbr\u003e\nAnimal free:No,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nSpecies:Human,\u003cbr\u003e\nStorage buffer:pH: 8Constituents: 0.79% Tris HCl, 0.3% Glutathione\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nShipped at conditions-Dry Ice, Appropriate short-term storage conditions--80°C, Appropriate long-term storage conditions--80°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nTafazzin also known as TAZ is a mitochondrial phospholipid-lysophospholipid transacylase enzyme. It plays a role in the remodeling of cardiolipin a phospholipid found predominantly in the mitochondrial inner membrane. Tafazzin has a molecular mass of approximately 33 kDa. It is expressed in various tissues with higher expression levels found in tissues with high energy demands such as the heart and skeletal muscles.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nTafazzin is essential for maintaining mitochondrial function and energy production. It is a critical component of the system responsible for cardiolipin metabolism. The enzyme does not work alone; it interacts with other mitochondrial proteins to facilitate the proper composition of cardiolipin species necessary for mitochondrial structural integrity and functionality.\u003cbr\u003e\nPathways\u003cbr\u003e\nTafazzin serves an important role in various lipid metabolism pathways. It participates in the phospholipid remodeling pathway where it interacts closely with acyltransferases to modify cardiolipin. This interaction is important for mitochondrial processes such as oxidative phosphorylation and energy conversion affecting proteins like cytochrome c oxidase and ATP synthase complex.\u003cbr\u003e\nTafazzin mutations are linked to Barth syndrome a rare genetic condition that affects the heart and muscles. This disorder is marked by symptoms including cardiomyopathy muscle weakness and neutropenia. Tafazzin dysfunction interrupts normal cardiolipin remodeling impacting proteins such as cytochrome c which contributes to abnormal energy metabolism seen in the syndrome. Additionally altered Tafazzin activity has connections to mitochondrial dysfunctions observed in some cardiomyopathies.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46843889352873,"sku":"ab114664","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab114664","provider":"Iright","version":"1.0","type":"link"}