IBA57 Antibody, HRP conjugated
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中文名稱：IBA57兔多克隆抗體, HRP偶聯
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貨號：CSB-PA722566LB01HU
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) IBA57 Polyclonal antibody
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Uniprot No.：
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基因名：IBA57
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別名：C1orf69 antibody; CAF17_HUMAN antibody; Chromosome 1 open reading frame 69 antibody; FLJ12734 antibody; FLJ13849 antibody; IBA57 antibody; IBA57 homolog iron sulfur cluster assembly antibody; IBA57 iron sulfur cluster assembly homolog antibody; IBA57 iron sulfur cluster assembly homolog (S. cerevisiae) antibody; IBA57; S. cerevisiae; homolog of antibody; Iron sulfur cluster assembly factor for biotin synthase and aconitase like antibody; iron-sulfur cluster assembly factor for biotin synthase- and aconitase-like mitochondrial proteins; with a mass of 57kDa antibody; Iron-sulfur cluster assembly factor homolog antibody; Mitochondrial proteins with a mass of 57kDa antibody; MMDS3 antibody; Putative transferase C1orf69 mitochondrial antibody; Putative transferase CAF17; mitochondrial antibody; SPG74 antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Putative transferase CAF17, mitochondrial protein (286-356AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：HRP
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G 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：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA
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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：Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway.
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基因功能參考文獻：
- a patient with infantile-onset optic atrophy and asymptomatic white matter involvement, thus broadening the phenotypic spectrum of biallelic IBA57 mutations. PMID: 30258207
- Description of four unrelated patients carrying novel mutations in IBA57; study expands the array of the genotypic variation of IBA57 and delineates the leukodystrophic pattern of IBA57 deficient patients PMID: 27785568
- Findings reinforce the suggested specific function of IBA57 in mitochondrial [4Fe-4S] protein maturation and provide additional evidence for its role in hereditary spastic paraplegia PMID: 25609768
- Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. PMID: 23462291
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相關疾?。?/div>Multiple mitochondrial dysfunctions syndrome 3 (MMDS3); Spastic paraplegia 74, autosomal recessive (SPG74)亞細胞定位：Mitochondrion.蛋白家族：GcvT family, CAF17 subfamily組織特異性：Expressed in skin fibroblasts and skeletal muscle (at protein level).數據庫鏈接：
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