ISCA1 Antibody, FITC conjugated

說明書

中文名稱：

ISCA1兔多克隆抗體, FITC偶聯
貨號：

CSB-PA887126LC01HU
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) ISCA1 Polyclonal antibody
Uniprot No.：

Q9BUE6
基因名：

ISCA1
別名：

GK004 antibody; HESB-like domain-containing protein 2 antibody; hIscA antibody; Iron sulfur assembly protein IscA antibody; Iron-sulfur assembly protein IscA antibody; Iron-sulfur cluster assembly 1 homolog antibody; Iron-sulfur cluster assembly 1 homolog, mitochondrial antibody; ISCA1 antibody; ISCA1_HUMAN antibody; mitochondrial antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Iron-sulfur cluster assembly 1 homolog, mitochondrial protein (67-122AA)
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. Probably involved in the binding of an intermediate of Fe/S cluster assembly.
基因功能參考文獻：
1. the phenotype observed in all affected subjects with the ISCA1 pathogenic variant is similar to that previously described in all four types of multiple mitochondrial dysfunctions syndromes. PMID: 28356563
2. The structural plasticity of the dimeric state of the [2Fe-2S] bound form of human GRX5 is the crucial factor that allows an efficient cluster transfer to the partner proteins human ISCA1 and ISCA2 by a specific protein-protein recognition mechanism. PMID: 24733926
3. The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity. PMID: 24217246
4. ISCA1, ISCA2, and IBA57 were depleted by RNA interference. Depleted cells contained massively swollen and enlarged mitochondria that were virtually devoid of cristae membranes, demonstrating the importance of these proteins for mitochondrial biogenesis. PMID: 22323289
5. Iron-binding activity of human iron-sulfur cluster assembly protein hIscA1 PMID: 20302570
6. screening a human brain cDNA expression library with serum from a patient suffering from the autoimmune Sjogren's syndrome yielded a gene product called hIscA implicated in iron-sulfur cluster biogenesis PMID: 15262227
7. Required for functional heme biosynthesis in erythroid cells. PMID: 19656490
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相關疾病：

Multiple mitochondrial dysfunctions syndrome 5 (MMDS5)
亞細胞定位：

Mitochondrion.
蛋白家族：

HesB/IscA family
組織特異性：

Detected in cerebellum, kidney and heart.
數據庫鏈接：

HGNC: 28660

OMIM: 611006

KEGG: hsa:81689

STRING: 9606.ENSP00000365159

UniGene: Hs.449291

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