ERAL1 Recombinant Monoclonal Antibody

中文名称：

ERAL1 Recombinant Monoclonal Antibody
货号：

CSB-RA007759MA1HU
规格：

￥1320
图片：
IHC image of CSB-RA007759MA1HU diluted at 1:200 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-human polymer IgG labeled by HRP and visualized using 0.05% DAB.
其他：

产品详情
产品评价
相关产品
靶点详情

产品详情

产品描述：

ERAL1 Recombinant Monoclonal Antibody（CSB-RA007759MA1HU）是一款针对线粒体核糖体组装因子ERAL1靶点开发的高特异性抗体。ERAL1作为线粒体核糖体小亚基的伴侣蛋白，在维持线粒体RNA稳定性及核糖体组装中发挥关键作用，其表达异常与能量代谢紊乱及细胞凋亡调控密切相关。本产品经严格验证，可精准识别内源性ERAL1蛋白，适用于ELISA和免疫组化（IHC）等实验，其中IHC推荐使用稀释比例为1:50-1:200，实验数据显示其在多种组织样本中呈现清晰的定位信号与低背景干扰。该抗体适用于探究线粒体功能调控机制、细胞代谢相关通路研究以及疾病模型中ERAL1表达水平变化的分析，为解析线粒体相关疾病的分子机制提供可靠工具。其稳定性和重复性已通过多批次质检验证，适用于基础科研领域中对线粒体核糖体组装、RNA加工及能量代谢过程的分子生物学研究。
Uniprot No.：

O75616
基因名：

ERAL1
别名：

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
反应种属：

Human
免疫原：

Recombinant Human ERAL1 protein
免疫原种属：

Homo sapiens (Human)
标记方式：

Non-conjugated
克隆类型：

Monoclonal
抗体亚型：

hIgG1
纯化方式：

Affinity-chromatography
克隆号：

27A9
浓度：

It differs from different batches. Please contact us to confirm it.
保存缓冲液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
产品提供形式：

Liquid
应用范围：

ELISA, IHC
推荐稀释比：

Application Recommended Dilution

IHC 1:50-1:200
Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
储存条件：

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

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.

产品评价

靶点详情

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.
基因功能参考文献：
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
相关疾病：

Perrault syndrome 6 (PRLTS6)
亚细胞定位：

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
数据库链接：

HGNC: 3424

OMIM: 607435

KEGG: hsa:26284

STRING: 9606.ENSP00000254928

UniGene: Hs.3426

ERAL1 Recombinant Monoclonal Antibody

产品详情

产品评价

相关产品

靶点详情

Most popular with customers

产品中心

信号通路

技术信息

联系信息