AUH Antibody
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中文名稱：AUH兔多克隆抗體
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貨號：CSB-PA002451GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：AUH
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別名：AUHMethylglutaconyl-CoA hydratase antibody; mitochondrial antibody; EC 4.2.1.18 antibody; AU-specific RNA-binding enoyl-CoA hydratase antibody; AU-binding protein/enoyl-CoA hydratase antibody; Itaconyl-CoA hydratase antibody; EC 4.2.1.56 antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human AUH
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.
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基因功能參考文獻：
- AUH localizes to the inner mitochondrial membrane and matrix where it associates with mitochondrial ribosomes and regulates protein synthesis. PMID: 24598254
- Phenotypic heterogeneity in two siblings with 3-methylglutaconic aciduria type I caused by a novel deletion of exons 1-3 within the AUH gene. PMID: 21840233
- 3-Methylglutaconic aciduria type I is caused by mutations in AUH PMID: 12434311
- Human 3-methylglutaconyl-CoA hydratase is identical with RNA-binding protein (AUH); molecular analyses of MGA1 patients show homozygosity or compound heterozygosity for mutations in AUH. PMID: 12655555
- Mutation analysis in the AUH gene revealed homozygosity for a novel splice site mutation IVS9-2A>G. We conclude that MGA1 may be associated with fever-associated seizures even in children without delayed psychomotor development. PMID: 15033206
- Mutations in the AUH gene are linked to metabolic disease 3-methylglutaconic aciduria type I (MGA1). PMID: 16640564
- The AUH trimer dimerizes upon binding to one molecule of a long RNA containing 24 repeats of the AUUU motif, (AUUU)(24)A. PMID: 18831052
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相關疾病：3-methylglutaconic aciduria 1 (MGA1)
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亞細胞定位：Mitochondrion.
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蛋白家族：Enoyl-CoA hydratase/isomerase family
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數據庫鏈接：
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