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  • 产品名称：anti-MMACHCantibody(MethylmalonicAciduria(CobalaminDeficiency)CblCType,withHomo

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简单介绍：
anti-MMACHCantibody(MethylmalonicAciduria(CobalaminDeficiency)CblCType,withHomocystinuria)(AA239-267)
详情介绍：
Immunogen This MMACHC antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 239-267 amino acids from the C-terminal region of human MMACHC.
Clone RB42667
Purification This antibody is purified through a protein A column, followed by peptide affinity purification. Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
Alternative Name MMACHC (MMACHC Antibody Abstract)
Background The exact function of the protein encoded by this gene is not known, however, its C-terminal region shows similarity to TonB, a bacterial protein involved in energy transduction for cobalamin (vitamin B12) uptake. Hence, it is postulated that this protein may have a role in the binding and intracellular trafficking of cobalamin. Mutations in this gene are associated with methylmalonic aciduria and homocystinuria type cblC.

Synonyms: MMACHC, Methylmalonic aciduria and homocystinuria type C protein
NCBI Accession NP_056321
UniProt Q9Y4U1
Research Area Signaling, Metabolism
Application Notes WB: 1:1000
Restrictions For Research Use only
Format Liquid
Concentration 0.39 mg/mL
Buffer Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
Preservative Sodium azide
Precaution of Use WARNING: Reagents contain sodium azide. Sodium azide is very toxic if ingested or inhaled. Avoid contact with skin, eyes, or clothing. Wear eye or face protection when handling. If skin or eye contact occurs, wash with copious amounts of water. If ingested or inhaled, contact a physician immediately. Sodium azide yields toxic hydrazoic acid under acidic conditions. Dilute azide-containing compounds in running water before discarding to avoid accumulation of potentially explosive deposits in lead or copper plumbing.
Supplier Images
Western Blotting (WB) image for anti-Methylmalonic Aciduria (Cobalamin Deficiency) CblC Type, with Homocystinuria (MMACHC) (AA 239-267), (C-Term) antibody (ABIN1881544) MMACHC Antibody (C-term) (ABIN1881544) western blot analysis in K562 cell line lysate...
Product cited in: Froese, Healy, McDonald, Kochan, Oppermann, Niesen, Gravel: "Thermolability of mutant MMACHC protein in the vitamin B12-responsive cblC disorder." in: Molecular genetics and metabolism, Vol. 100, Issue 1, pp. 29-36, 2010 (PubMed).

Davila, Froeling, Tan, Bonnard, Boland, Snippe, Hibberd, Seielstad: "New genetic associations detected in a host response study to hepatitis B vaccine." in: Genes and immunity, Vol. 11, Issue 3, pp. 232-8, 2010 (PubMed).

Profitlich, Kirmse, Wasserstein, Diaz, Srivastava: "High prevalence of structural heart disease in children with cblC-type methylmalonic aciduria and homocystinuria." in: Molecular genetics and metabolism, Vol. 98, Issue 4, pp. 344-8, 2009 (PubMed).

Kim, Hannibal, Gherasim, Jacobsen, Banerjee: "A human vitamin B12 trafficking protein uses glutathione transferase activity for processing alkylcobalamins." in: The Journal of biological chemistry, Vol. 284, Issue 48, pp. 33418-24, 2009 (PubMed).

Richard, Jorge-Finnigan, Garcia-Villoria, Merinero, Desviat, Gort, Briones, Leal, Pérez-Cerdá, Ribes, Ugarte, Pérez: "Genetic and cellular studies of oxidative stress in methylmalonic aciduria (MMA) cobalamin deficiency type C (cblC) with homocystinuria (MMACHC)." in: Human mutation, Vol. 30, Issue 11, pp. 1558-66, 2009 (PubMed).