{"product_id":"anti-mecr-antibody-picoband-r","title":"Anti-MECR Antibody Picoband®","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100 μg\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e: At -20℃ for one year from date of receipt. \nAfter reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months.\nAvoid repeated freezing and thawing.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e: Lyophilized\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e: Human,Mouse,Rat\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e: WB,IHC,Flow Cytometry,ELISA\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplication Details\u003c\/b\u003e: Western blot, 0.25-0.5 μg\/ml, Human, Mouse, Rat\u003cbr\u003e\nImmunohistochemistry(Paraffin-embedded Section), 1-2 μg\/ml, Human\u003cbr\u003e\nFlow Cytometry (Fixed), 1-3 μg\/1x10\u003csup\u003e6\u003c\/sup\u003e cells, Human\u003cbr\u003e\nELISA, 0.1-0.5 μg\/ml, -\u003cbr\u003e\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Name\u003c\/b\u003e: MECR\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecificity\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: Trans-2-enoyl-CoA reductase, mitochondrial is an enzyme that in humans is encoded by the MECR gene. The protein encoded by this gene is an oxidoreductase that catalyzes the last step in mitochondrial fatty acid synthesis. Defects in this gene are a cause of childhood-onset dystonia and optic atrophy.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e: E.coli-derived human MECR recombinant protein (Position: E39-A359).\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e: Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eContents\u003c\/b\u003e: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification\u003c\/b\u003e: Immunogen affinity purified.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReconstitution\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReference\u003c\/b\u003e: 1. Heimer, G., Keratar, J. M., Riley, L. G., Balasubramaniam, S., Eyal, E., Pietikainen, L. P., Hiltunen, J. K., Marek-Yagel, D., Hamada, J., Gregory, A., Rogers, C., Hogarth, P., and 24 others. MECR mutations cause childhood-onset dystonia and optic atrophy, a mitochondrial fatty acid synthesis disorder. Am. J. Hum. Genet. 99: 1229-1244, 2016.\n2. Liu, Z., Shimura, M., Zhang, L., Zhang, W., Wang, J., Ogawa-Tominaga, M., Wang, J., Wang, X., Lv, J., Shi, W., Zhang, V. W., Murayama, K., Fang, F. Whole exome sequencing identifies a novel homozygous MECR mutation in a Chinese patient with childhood-onset dystonia and basal ganglia abnormalities, without optic atrophy. Mitochondrion 57: 222-229, 2021. \n3. Miinalainen, I. J., Chen, Z.-J., Torkko, J. M., Pirila, P. L., Sormunen, R. T., Bergmann, U., Qin, Y.-M., Hiltunen, J. K. Characterization of 2-enoyl thioester reductase from mammals: an ortholog of Ybr026p\/Mrf1'p of the yeast mitochondrial fatty acid synthesis type II. J. Biol. Chem. 278: 20154-20161, 2003.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniprot ID\u003c\/b\u003e: Q9BV79\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHost\u003c\/b\u003e: Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConcentration\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eCross Reactivity\u003c\/b\u003e: No cross-reactivity with other proteins.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e: Rabbit IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePhospho_site\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eObserved Molecular Weight\u003c\/b\u003e: 38 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eCalculated Molecular Weight\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene ID\u003c\/b\u003e: 51102\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProtein Name\u003c\/b\u003e: Enoyl-[acyl-carrier-protein] reductase, mitochondrial\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Full Name\u003c\/b\u003e: mitochondrial trans-2-enoyl-CoA reductase\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e: CGI 63; CGI-63; FASN2B; HsNrbf 1; MECR; NBRF1; NRBF 1; NRBF1; HsNrbf-1; NRBF-1\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48787929858148,"sku":"A08189-1","price":431.0,"currency_code":"GBP","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_069a2fcd-711e-44cc-a629-4dce3fedbda2.jpg?v=1784405484","url":"https:\/\/www.genebiosystems.com\/en-gb\/products\/anti-mecr-antibody-picoband-r","provider":"GeneBio ","version":"1.0","type":"link"}