{"product_id":"human-methylcrotonoyl-coa-carboxylase-beta-chain-mitochondrial-mccc2-elisa-kit-bhe13703911","title":"Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2) ELISA Kit","description":"\u003cp\u003e\u003cb\u003eFeatures \u0026amp; Benefits\u003c\/b\u003e\u003c\/p\u003e\u003cp\u003eHuman Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2) ELISA Kit has high sensitivity and excellent specificity for detection of Human MCCC2. No significant cross-reactivity or interference between Human MCCC2 and analogues was observed.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e\u003c\/p\u003e\u003cp\u003eMCCC2 is the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) . MCC2 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3- hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.\u003c\/p\u003e","brand":"Abbkine Scientific Co., Ltd.","offers":[{"title":"48 T","offer_id":53338639466861,"sku":"KTE61696-48T","price":339.0,"currency_code":"USD","in_stock":true},{"title":"96 T","offer_id":53338842857837,"sku":"KTE61696-96T","price":589.0,"currency_code":"USD","in_stock":true},{"title":"96 T×5","offer_id":53338842890605,"sku":"KTE61696-96TX5","price":2629.0,"currency_code":"USD","in_stock":true},{"title":"96 T×50","offer_id":53338842923373,"sku":"KTE61696-96TX50","price":24569.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/KTE61696.png?v=1778954783","url":"https:\/\/www.ebiohippo.com\/products\/human-methylcrotonoyl-coa-carboxylase-beta-chain-mitochondrial-mccc2-elisa-kit-bhe13703911","provider":"BioHippo","version":"1.0","type":"link"}