GUF1 Antibody

說明書

產品詳情

Uniprot No.：

Q8N442
基因名：

GUF1
別名：

GUF1 antibody; Translation factor GUF1 antibody; mitochondrial antibody; EC 3.6.5.- antibody; Elongation factor 4 homolog antibody; EF-4 antibody; GTPase GUF1 antibody; Ribosomal back-translocase antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human GUF1
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IF
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunofluorescence (IF) 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：

Promotes mitochondrial protein synthesis. May act as a fidelity factor of the translation reaction, by catalyzing a one-codon backward translocation of tRNAs on improperly translocated ribosomes. Binds to mitochondrial ribosomes in a GTP-dependent manner.
基因功能參考文獻：
1. Homozygous variant in the GUF1 gene identified in the three siblings with West syndrome. PMID: 26486472
2. The structure provides insights into the tRNA-remodeling function of EF-4 on the ribosome and suggests that the displacement of the CCA-end of the A-site tRNA away from the peptidyl transferase center (PTC) is functionally significant. PMID: 27092003
相關疾病：

Epileptic encephalopathy, early infantile, 40 (EIEE40)
亞細胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

TRAFAC class translation factor GTPase superfamily, Classic translation factor GTPase family, LepA subfamily
數據庫鏈接：

HGNC: 25799

OMIM: 617064

KEGG: hsa:60558

STRING: 9606.ENSP00000281543

UniGene: Hs.546419

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