GUF1 Antibody

產(chǎn)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(yīng)種屬：

Human
免疫原：

Synthesized peptide derived from the Internal region of Human GUF1.
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度：

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

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

IHC, ELISA

推薦稀釋比：

Application	Recommended Dilution
IHC	1:100-1:300
ELISA	1:20000

Protocols：

Immunohistochemistry (IHC) Protocol
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.

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(xiàn)：
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(guān)疾病：

Epileptic encephalopathy, early infantile, 40 (EIEE40)
亞細(xì)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(shù)據(jù)庫鏈接：

HGNC: 25799

OMIM: 617064

KEGG: hsa:60558

STRING: 9606.ENSP00000281543

UniGene: Hs.546419