{"product_id":"new-product-402355","title":"SURF1 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":"Bioworld","offers":[{"title":"100ul \/ Rabbit \/ Human,Mouse,Rat","offer_id":44092413640928,"sku":"BS71913","price":408.0,"currency_code":"KRW","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0601\/9330\/8896\/products\/bioWORLD-Logo-New_1617713956_fde48a4e-b1fb-4687-aa93-dd9c6887ea13.png?v=1689728017","url":"https:\/\/rndmate.com\/products\/new-product-402355","provider":"알앤디메이트","version":"1.0","type":"link"}