Description
| 抗体名: | EIF2B3 Antibody (PACO06454) |
| 抗体コード: | PACO06454 |
| サイズ: | 50ug |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB |
| 推奨される希釈: | ELISA:1:5000, WB:1:500-1:2000 |
| 反応性: | Human, Mouse |
| 免疫原: | Synthesized peptide derived from the Internal region of human eIF2B γ . |
| 憲法: | Liquid |
| ストレージバッファ: | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| 精製方法: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
 | Western Blot analysis of K562 cells using eIF2B gamma Polyclonal Antibody. |
 | Western Blot analysis of K562 cells using eIF2B gamma Polyclonal Antibody. |
| シノニム: | EIF2B3; Translation initiation factor eIF-2B subunit γ; eIF-2B GDP-GTP exchange factor subunit γ |
| UniProt Protein Function: | eIF2B-gamma: Catalyzes the exchange of eukaryotic initiation factor 2-bound GDP for GTP. Defects in EIF2B3 are a cause of leukodystrophy with vanishing white matter (VWM). VWM is a leukodystrophy that occurs mainly in children. Neurological signs include progressive cerebellar ataxia, spasticity, inconstant optic atrophy and relatively preserved mental abilities. The disease is chronic-progressive with, in most individuals, additional episodes of rapid deterioration following febrile infections or minor head trauma. While childhood onset is the most common form of the disorder, some severe forms are apparent at birth. A severe, early-onset form seen among the Cree and Chippewayan populations of Quebec and Manitoba is called Cree leukoencephalopathy. Milder forms may not become evident until adolescence or adulthood. Some females with milder forms of the disease who survive to adolescence exhibit ovarian dysfunction. This variant of the disorder is called ovarioleukodystrophy. Belongs to the eIF-2B gamma/epsilon subunits family. 3 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Translation; Translation initiation Chromosomal Location of Human Ortholog: 1p34.1 Cellular Component: cytoplasm; cytosol; eukaryotic translation initiation factor 2B complex Molecular Function:guanyl-nucleotide exchange factor activity; protein binding; translation factor activity, nucleic acid binding; translation initiation factor activity Biological Process: cellular response to stimulus; negative regulation of translation initiation in response to stress; oligodendrocyte development; response to glucose stimulus; response to heat; response to peptide hormone stimulus; translational initiation Disease: Leukoencephalopathy With Vanishing White Matter |
| NCBI Summary: | The protein encoded by this gene is one of the subunits of initiation factor eIF2B, which catalyzes the exchange of eukaryotic initiation factor 2-bound GDP for GTP. It has also been found to function as a cofactor of hepatitis C virus internal ribosome entry site-mediated translation. Mutations in this gene have been associated with leukodystrophy with vanishing white matter. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009] |
| UniProt Code: | Q9NR50 |
| NCBI GenInfo Identifier: | 18203317 |
| NCBI Gene ID: | 8891 |
| NCBI Accession: | Q9NR50.1 |
| UniProt Secondary Accession: | Q9NR50,Q5QP89, Q5QP90, Q8NDB5, Q8WV57, Q9H850, B2RBH8 D3DPZ2, |
| UniProt Related Accession: | Q9NR50 |
| Molecular Weight: | 50kDa |
| NCBI Full Name: | Translation initiation factor eIF-2B subunit gamma |
| NCBI Synonym Full Names: | eukaryotic translation initiation factor 2B subunit gamma |
| NCBI Official Symbol: | EIF2B3 |
| NCBI Official Synonym Symbols: | EIF-2B; EIF2Bgamma |
| NCBI Protein Information: | translation initiation factor eIF-2B subunit gamma |
| UniProt Protein Name: | Translation initiation factor eIF-2B subunit gamma |
| UniProt Synonym Protein Names: | eIF-2B GDP-GTP exchange factor subunit gamma |
| Protein Family: | Translation initiation factor |
| UniProt Gene Name: | EIF2B3 |
