ERAL1 Antibody, Biotin conjugated

說明書

中文名稱：

ERAL1兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA007759LD01HU
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) ERAL1 Polyclonal antibody
Uniprot No.：

O75616
基因名：

ERAL1
別名：

CEGA antibody; Conserved ERA like GTPase antibody; Conserved ERA-like GTPase antibody; ERA antibody; Era (E. coli G protein homolog) like 1 antibody; Era G protein like 1 (E. coli) antibody; Era G protein like 1 antibody; ERA W antibody; ERA-like protein 1 antibody; ERA-W antibody; Eral1 antibody; ERAL1_HUMAN antibody; ERAL1A antibody; ERAW antibody; GTP binding protein era homolog antibody; GTPase Era antibody; GTPase Era; mitochondrial antibody; GTPase human homolog of E. coli essential cell cycle protein Era antibody; H-ERA antibody; HERA A antibody; hERA antibody; HERA B antibody; HERAA antibody; HERAB antibody; mitochondrial antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human GTPase Era, mitochondrial protein (271-382AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
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
Protocols：

ELISA 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.

產品評價

靶點詳情

功能：

Probable GTPase that plays a role in the mitochondrial ribosomal small subunit assembly. Specifically binds the 12S mitochondrial rRNA (12S mt-rRNA) to a 33 nucleotide section delineating the 3' terminal stem-loop region. May act as a chaperone that protects the 12S mt-rRNA on the 28S mitoribosomal subunit during ribosomal small subunit assembly.
基因功能參考文獻：
1. Three women from three different families presented with signs of PS, including deafness and ovarian dysgenesis. We employed exome sequencing and identified a homozygous missense mutation in the ERAL1 gene, c.707A > T. The mutation leads to a damaging p.Asn236Ile substitution that is likely to interfere with the GTP binding capacity of the ERAL1 protein. PMID: 28449065
2. Data show that loss of ERAL1 leads to rapid decay of nascent 12S mt-rRNA, consistent with a role as a mitochondrial RNA chaperone. PMID: 20604745
相關疾病：

Perrault syndrome 6 (PRLTS6)
亞細胞定位：

Mitochondrion matrix. Mitochondrion inner membrane; Peripheral membrane protein. Note=Localizes on the matrix side on the mitochondrial inner membrane.
蛋白家族：

TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, Era GTPase family
數據庫鏈接：

HGNC: 3424

OMIM: 607435

KEGG: hsa:26284

STRING: 9606.ENSP00000254928

UniGene: Hs.3426

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