{"product_id":"abcam-ab305255","title":"Abcam, ab305255, Anti-SPG7\/PGN antibody [EPR26225-9]","description":"\u003cp\u003eSize: 20µL \/ 100µL \/ 1mL\u003cbr\u003e\nRabbit Recombinant Monoclonal SPG7\/PGN antibody. Suitable for WB, IP and reacts with Human, Mouse, Rat samples.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:EPR26225-9,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Human, Mouse, Rat,\u003cbr\u003e\nApplications:IP, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:The exact immunogen used to generate this antibody is proprietary information.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nPatented technology\u003cbr\u003e\nOur RabMAb\u003cbr\u003e\ntechnology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to\u003cbr\u003e\nRabMAb® patents\u003cbr\u003e\nWhat are the advantages of a recombinant monoclonal antibody?\u003cbr\u003e\nThis product is a recombinant monoclonal antibody, which offers several advantages including:\u003cbr\u003e\n- High batch-to-batch consistency and reproducibility\u003cbr\u003e\n- Improved sensitivity and specificity\u003cbr\u003e\n- Long-term security of supply\u003cbr\u003e\n- Animal-free batch production\u003cbr\u003e\nFor more information, read more on\u003cbr\u003e\nrecombinant antibodies\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.2 - 7.4Preservative: 0.01% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 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":46850353365161,"sku":"ab305255","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab305255","provider":"Iright","version":"1.0","type":"link"}