{"product_id":"new-product-272991","title":"SURF-1 (F181) polyclonal antibody","description":"\u003cp\u003eThe SURF-1 protein demonstrates a vital role in the assembly of complex IV (CIV or COX) of the mitochondrial respiratory chain. Expressed in the inner mitochondrial membrane, mutations of the SURF-1 gene generally cause cytochrome c oxidase complex IV deficiency. Shortage of complex IV leads to Leigh syndrome, a severe neurological disorder. Leigh syndrome patients are usually subject to rapidly progressive encephalopathy, characterized by necrotic lesions in subcortical brain regions. SURF-1 mutations correlate to high post-implantation embryonic lethality as well as early-onset mortality of post-natal individuals. Considerable deficit in muscle strength and motor performance is also a profound and isolated defect of SURF-1 activity in skeletal muscle and liver. Heart, brain and skeletal muscle morphological abnormalities frequently occur due to SURF-1 mutations.\u003c\/p\u003e","brand":"ABCbiolab","offers":[{"title":"100ul \/ Rabbit \/ Human,Mouse,Rat","offer_id":45803303698712,"sku":"ABCBW01081","price":325.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/758-7580448_monoclonal-antibody-png-transparent-png_4bd6b618-bdf5-4138-b967-d1673d19f0c0.png?v=1689734083","url":"https:\/\/danabiosci.com\/products\/new-product-272991","provider":"Dana Bioscience","version":"1.0","type":"link"}