{"product_id":"new-product-223594","title":"PDHA1 polyclonal antibody","description":"\u003cp\u003eThe pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The E1 enzyme of the PDH complex is made up of a heterotetramer of two α and two β subunits. The E1-α subunit (PDH-E1α) contains the E1 active site and plays a key role in the function of the PDH complex. The PDH complex is regulated by phosphorylation and dephosphorylation of PDH-E1α. The gene encoding for PDH-E1α maps to chromosome Xp22.12, and a 20bp deletion in the last exon of this gene is sufficient to cause PDH deficiency, which causes a broad range of symptoms including the development of seizures, mental retardation and spasticity, as well as intermittent episodes of lactic acidosis associated with cerebellar ataxia.\u003c\/p\u003e","brand":"Bioworld","offers":[{"title":"100ul \/ Rabbit \/ Human, Mouse, Rat","offer_id":47661594018072,"sku":"BS91040","price":448.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/BIOWORLD_810e9432-9802-4ce3-b81b-aa413df78dda.jpg?v=1707460311","url":"https:\/\/danabiosci.com\/products\/new-product-223594","provider":"Dana Bioscience","version":"1.0","type":"link"}