{"product_id":"abcam-ab317174","title":"Abcam, ab317174, Anti-SPG7 antibody [HL2236]","description":"\u003cp\u003eSize: 100µL\u003cbr\u003e\nRabbit Monoclonal SPG7\/PGN antibody. Suitable for ICC\/IF, WB and reacts with Human, Mouse, Rat samples. Immunogen corresponding to Recombinant Fragment Protein within Human SPG7.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:HL2236,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Human, Mouse, Rat,\u003cbr\u003e\nApplications:WB, ICC\/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Recombinant Fragment Protein within Human SPG7.Q9UQ90\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Storage buffer-pH: 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°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\nSPG7 also known as paraplegin is a mitochondrial metalloprotease. It has a molecular mass of approximately 88 kDa. SPG7 is encoded by the SPG7 gene located in humans on chromosome 16. It expresses widely in tissues with a strong presence in the nervous system and muscles. Alternate names for this protein are PGN and spastic paraplegia 7 due to its association with specific neurodegenerative conditions.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nSPG7 functions as a part of the mitochondrial inner membrane m-AAA protease complex. This complex performs essential roles in the maintenance of mitochondrial homeostasis by degrading damaged or misfolded proteins within the mitochondria. The presence of SPG7 is important for proper mitochondrial function. The protein has proteolytic activity which supports mitochondrial respiratory chain complexes' assembly and stability.\u003cbr\u003e\nPathways\u003cbr\u003e\nSPG7 directly involves itself in mitochondrial protein quality control and the broader regulation of mitochondrial function. It interacts closely with ATP-dependent proteases like AFG3L2 playing a significant part in mitochondrial biogenesis pathways. These partnerships are key for maintaining cellular energy balance and ensuring efficient electron transport chain operation.\u003cbr\u003e\nSPG7 mutations connect predominantly to hereditary spastic paraplegia (HSP) and have also been linked to ataxia. These mutations interrupt normal mitochondrial function leading to the degeneration of motor pathways. In HSP SPG7 interacts with proteins such as paraplegin-associated proteins SPG5 and SPG11 contributing to the disorder's pathogenesis. Understanding SPG7’s role in these diseases aids in exploring therapeutic strategies for mitochondrial-related disorders.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46850343272617,"sku":"ab317174","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab317174","provider":"Iright","version":"1.0","type":"link"}