产品货号 : mlR12680
英文名称 : ValRS
中文名称 : 缬氨酰-tRNA合成酶1抗体
别 名 : Bat 6; Bat6; D17H6S56E; EC 6.1.1.9; G7A; Protein G7a; SYVC_HUMAN; valine tRNA ligase 1, cytoplasmic; Valine tRNA ligase; Valine--tRNA ligase; ValRS; VALYL tRNA SYNTHETASE 1; valyl tRNA synthetase 2; valyl-tRNA synthetase 2; Valyl-tRNA synthetase; VARS; Vars1; VARS2; VARS2, formerly.
研究领域 : 细胞生物 转录调节因子 表观遗传学
抗体来源 : Rabbit
克隆类型 : Polyclonal
交叉反应 : Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Danio
产品应用 : ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 : 140kDa
细胞定位 : 细胞浆
性 状 : Lyophilized or Liquid
浓 度 : 1mg/ml
免 疫 原 : KLH conjugated synthetic peptide derived from human ValRS:401-500/1264
亚 型 : IgG
纯化方法 : affinity purified by Protein A
储 存 液 : 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 : Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed : PubMed
产品介绍 : Aminoacyl-tRNA synthetases catalyze the aminoacylation of tRNA by their cognate amino acid. Because of their central role in linking amino acids with nucleotide triplets contained in tRNAs, aminoacyl-tRNA synthetases are thought to be among the first proteins that appeared in evolution. The protein encoded by this gene belongs to class-I aminoacyl-tRNA synthetase family and is located in the class III region of the major histocompatibility complex. [provided by RefSeq, Jul 2008]
DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A disorder due to mitochondrial respiratory chain complex defects. Clinical features are variable and include muscle weakness with hypotonia, central neurological disease with progressive external ophthalmoplegia, ptosis and ataxia, delayed psychomotor development, cardiomyopathy, abnormal liver function, facial dysmorphism, microcephaly and epilepsy.
Similarity:
Belongs to the class-I aminoacyl-tRNA synthetase family.
Contains 1 GST C-terminal domain.
SWISS:
P26640
Gene ID:
7407
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.