{"product_id":"anti-hccs-antibody-picoband-r","title":"Anti-HCCS Antibody Picoband®","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100 μg\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.\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,ICC,IF,Flow Cytometry,ELISA\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplication Details\u003c\/b\u003e: Western blot, 0.25-0.5μg\/ml, Human, Mouse, Rat\u003cbr\u003e\nImmunohistochemistry (Paraffin-embedded Section), 2-5μg\/ml, Human, Rat\u003cbr\u003e\nImmunocytochemistry\/Immunofluorescence, 5μg\/ml, Human\u003cbr\u003e\nFlow Cytometry (Fixed), 1-3μg\/1x10\u003csup\u003e6\u003c\/sup\u003e cells, Human\u003cbr\u003e\nELISA, 0.1-0.5μg\/ml, -\u003cbr\u003e\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Name\u003c\/b\u003e: HCCS\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecificity\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: Cytochrome c-type heme lyase is an enzyme that in humans is encoded by the HCCS gene on chromosome X. The protein encoded by this gene is an enzyme that covalently links a heme group to the apoprotein of cytochrome c. Defects in this gene are a cause of microphthalmia syndromic type 7 (MCOPS7). Three transcript variants encoding the same protein have been found for this gene.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e: E.coli-derived human HCCS recombinant protein (Position: K76-S268).\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e: Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eContents\u003c\/b\u003e: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4.\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification\u003c\/b\u003e: Immunogen affinity purified.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReconstitution\u003c\/b\u003e: Add 0.2ml of distilled water will yield a concentration of 500ug\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReference\u003c\/b\u003e: 1. Indrieri, A., Conte, I., Chesi, G., Romano, A., Quartararo, J., Tate, R., Ghezzi, D., Zeviani, M., Goffrini, P., Ferrero, I., Bovolenta, P., Franco, B. The impairment of HCCS leads to MLS syndrome by activating a non-canonical cell death pathway in the brain and eyes. EMBO Molec. Med. 5: 280-293, 2013. Note: Erratum: EMBO Molec. Med. 6: 849 only, 2014. \n2. Morleo, M., Pramparo, T., Perone, L., Gregato, G., Le Caignec, C., Mueller, R. F., Ogata, T., Raas-Rothschild, A., de Blois, M. C., Wilson, L. C., Zaidman, G., Zuffardi, O., Ballabio, A., Franco, B. Microphthalmia with linear skin defects (MLS) syndrome: clinical, cytogenetic, and molecular characterization of 11 cases. Am. J. Med. Genet. 137A: 190-198, 2005. \n3. Prakash, S. K., Cormier, T. A., McCall, A. E., Garcia, J. J., Sierra, R., Haupt, B., Zoghbi, H. Y., Van den Veyver, I. B. Loss of holocytochrome c-type synthetase causes the male lethality of X-linked dominant microphthalmia with linear skin defects (MLS) syndrome. Hum. Molec. Genet. 11: 3237-3248, 2002.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniprot ID\u003c\/b\u003e: P53701\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: 31 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eCalculated Molecular Weight\u003c\/b\u003e: 27904 MW\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene ID\u003c\/b\u003e: 3052\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProtein Name\u003c\/b\u003e: Holocytochrome c-type synthase\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Full Name\u003c\/b\u003e: holocytochrome c synthase\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e: CCHL; Cytochrome c type heme lyase; HCCS; Holocytochrome c type synthase; MCOPS7\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48787698843748,"sku":"A00924-2","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_9f0804ec-be47-430c-9611-ea67837af3c9.jpg?v=1784402577","url":"https:\/\/www.genebiosystems.com\/en-gb\/products\/anti-hccs-antibody-picoband-r","provider":"GeneBio ","version":"1.0","type":"link"}