{"product_id":"human-methylcrotonoyl-coa-carboxylase-beta-chain-mitochondrial-mccc2-elisa-kit-bhe11404622","title":"Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2) ELISA Kit","description":"\u003cdiv class=\"bhp-desc\"\u003e\n\u003cstyle\u003e\n.bhp-desc{font-size:16px;color:#1a1a1a;line-height:1.7}\n.bhp-desc h2{font-size:18px;font-weight:700;color:#003366;margin:24px 0 10px;padding-bottom:6px;border-bottom:2px solid #003366}\n.bhp-desc p{margin:0 0 12px}\n.bhp-desc .bhp-spec-table{width:100%;border-collapse:collapse;margin:12px 0 18px}\n.bhp-desc .bhp-spec-table th{width:36%;text-align:left;padding:8px 12px;background:#e8f0fb;color:#003366;font-weight:600;vertical-align:top;border:1px solid #ccd9f0}\n.bhp-desc .bhp-spec-table td{padding:8px 12px;vertical-align:top;border:1px solid #ccd9f0}\n.bhp-desc .bhp-qc-badge{display:inline-block;background:#e6f4ea;color:#1a7a3c;border:1px solid #a8d5b5;border-radius:4px;padding:6px 14px;font-size:14px;font-weight:600}\n.bhp-desc .bhp-warning-box{background:#fff8e6;border-left:4px solid #e6a817;padding:10px 14px;margin:10px 0 14px;border-radius:0 4px 4px 0}\n\u003c\/style\u003e\n\u003ch2\u003eScientific Background\u003c\/h2\u003e\n\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 asymptomat.\u003c\/p\u003e\n\u003ch2\u003eAssay Principle\u003c\/h2\u003e\n\u003cp\u003eThis assay employs a two-site sandwich ELISA to quantitate MCCC2 in samples. An antibody specific for MCCC2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyMCCC2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MCCC2 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of MCCC2 bound in the initial step. The color development is stopped and the intensity of the color is measured.\u003c\/p\u003e\n\u003ch2\u003ePerformance Specifications\u003c\/h2\u003e\n\u003ctable class=\"bhp-spec-table\"\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003cth\u003eSensitivity\u003c\/th\u003e\n\u003ctd\u003e0.141 ng\/mL\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eDetection Range\u003c\/th\u003e\n\u003ctd\u003e0.312-20 ng\/mL\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eTotal Assay Time\u003c\/th\u003e\n\u003ctd\u003e3.5–5 hours\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eCompatible Sample Types\u003c\/th\u003e\n\u003ctd\u003eserum, plasma, cell culture supernatant, tissue homogenate\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eSpecies Reactivity\u003c\/th\u003e\n\u003ctd\u003eHuman\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eDetection Method\u003c\/th\u003e\n\u003ctd\u003eColorimetric (TMB\/HRP)\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth\u003eStorage\u003c\/th\u003e\n\u003ctd\u003e4°C (short-term); -20°C (long-term)\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e\n\u003cp\u003e\u003cspan class=\"bhp-qc-badge\"\u003e✓ Research-Grade Validation\u003c\/span\u003e\u003c\/p\u003e\n\u003ch2\u003eSafety \u0026amp; Regulatory\u003c\/h2\u003e\n\u003cdiv class=\"bhp-warning-box\"\u003e\n\u003cstrong\u003eResearch Use Only (RUO).\u003c\/strong\u003e This product is intended for research purposes only and is not approved for diagnostic, therapeutic, or clinical use.\u003c\/div\u003e\n\u003cp\u003eHandle reagents in accordance with institutional biosafety guidelines. Refer to the Safety Data Sheet (SDS) for complete hazard and handling information. Contains components that may require special disposal procedures per local regulations.\u003c\/p\u003e\n\u003c\/div\u003e","brand":"Wuhan Abebio Science Co Ltd","offers":[{"title":"48 T","offer_id":53337725305197,"sku":"AE34053HU-48T","price":325.0,"currency_code":"USD","in_stock":true},{"title":"96 T","offer_id":53337890619757,"sku":"AE34053HU-96T","price":610.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/1_1dc82338-3f86-4df0-aa86-b4ead7396351.jpg?v=1778952132","url":"https:\/\/www.ebiohippo.com\/products\/human-methylcrotonoyl-coa-carboxylase-beta-chain-mitochondrial-mccc2-elisa-kit-bhe11404622","provider":"BioHippo","version":"1.0","type":"link"}