IBA57 Antibody, FITC conjugated

說明書

中文名稱：

IBA57兔多克隆抗體, FITC偶聯
貨號：

CSB-PA722566LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) IBA57 Polyclonal antibody
Uniprot No.：

Q5T440
基因名：

IBA57
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Putative transferase CAF17, mitochondrial protein (286-356AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
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
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway.
基因功能參考文獻：
1. a patient with infantile-onset optic atrophy and asymptomatic white matter involvement, thus broadening the phenotypic spectrum of biallelic IBA57 mutations. PMID: 30258207
2. 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
3. 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
4. Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. PMID: 23462291
相關疾病：

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：

HGNC: 27302

OMIM: 615316

KEGG: hsa:200205

STRING: 9606.ENSP00000355672

UniGene: Hs.237017

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