BOLA3 Antibody

中文名稱：

BOLA3兔多克隆抗體
貨號：

CSB-PA690486LA01HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA690486LA01HU at dilution of 1:100
其他：

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) BOLA3 Polyclonal antibody
Uniprot No.：

Q53S33
基因名：

BOLA3
別名：

BolA-like protein 3 antibody; BOLA3 antibody; BOLA3_HUMAN antibody; MMDS2 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human BolA-like protein 3 protein (1-79AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，BOLA3 Antibody (CSB-PA690486LA01HU)，的標記方式是Non-conjugated。對于BOLA3 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA690486LB01HU	BOLA3 Antibody, HRP conjugated	ELISA
FITC	CSB-PA690486LC01HU	BOLA3 Antibody, FITC conjugated
Biotin	CSB-PA690486LD01HU	BOLA3 Antibody, Biotin conjugated	ELISA

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
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
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.

功能：

Acts as a mitochondrial iron-sulfur (Fe-S) cluster assembly factor that facilitates (Fe-S) cluster insertion into a subset of mitochondrial proteins. Probably acts together with NFU1.
基因功能參考文獻：
1. Clinical features of BOLA3-associated variant nonketotic hyperglycemia include severe neurodegeneration after a period of normal development. PMID: 24334290
2. BOLA3 plays a crucial role in the biogenesis of iron-sulfur clusters. PMID: 22562699
3. Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes [case report] PMID: 21944046
4. Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator) PMID: 20379614
5. This study reported that all three human BolA proteins (hBolA1, hBolA2, and hBolA3) are novel non-classical secreted proteins identified with bioinformatics and molecular biology experiments. PMID: 18548201
6. BOLA-like proteins are widely conserved from prokaryotes to eukaryotes and may be involved in cell proliferation or cell-cycle regulation. PMID: 14718656
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相關疾病：

Multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia (MMDS2)
亞細胞定位：

Mitochondrion.
蛋白家族：

BolA/IbaG family
組織特異性：

Widely expressed.
數據庫鏈接：

HGNC: 24415

OMIM: 613183

KEGG: hsa:388962

STRING: 9606.ENSP00000331369

UniGene: Hs.61472

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