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货号： bs-20281R-AF594 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-20281R-AF594
- 英文名称
- Anti-HARS/AF594
- 中文名称
- AF594标记的组氨酸tRNA连接酶抗体
- 别 名
- EC 6.1.1.21; FLJ20491; HisRS; Jo-1; histidine translase; Histidine tRNA ligase; Histidyl tRNA synthetase; HRS; Human histidyl tRNA synthetase homolog (HO3) mRNA complete cds; SYHC_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 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, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 57kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human HARS
- 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.
- 产品介绍
- background:
Aminoacyl-tRNA synthetases are a class of enzymes thatcharge tRNAs with their cognate amino acids. The protein encoded bythis gene is a cytoplasmic enzyme which belongs to the class IIfamily of aminoacyl-tRNA synthetases. The enzyme is responsible forthe synthesis of histidyl-transfer RNA, which is essential for theincorporation of histidine into proteins. The gene is located in ahead-to-head orientation with HARSL on chromosome five, where thehomologous genes share a bidirectional promoter. The gene productis a frequent target of autoantibodies in the human autoimmunedisease polymyositis/dermatomyositis. Several transcript variantsencoding different isoforms have been found for this gene.
Subcellular Location:
Cytoplasmic
Tissue Specificity:
Brain, heart, liver and kidney.
Post-translational modifications:
Defects in HARS are a cause of Usher syndrome type 3B(USH3B) [MIM:614504]. USH3B is a syndrome characterized byprogressive vision and hearing loss during early childhood. Somepatients have the so-called Charles Bonnet syndrome, involvingdecreased visual acuity and vivid visual hallucinations. USH is agenetically heterogeneous condition characterized by theassociation of retinitis pigmentosa with sensorineural deafness.Age at onset and differences in auditory and vestibular functiondistinguish Usher syndrome type 1 (USH1), Usher syndrome type 2(USH2) and Usher syndrome type 3 (USH3). USH3 is characterized bypostlingual, progressive hearing loss, variable vestibulardysfunction, and onset of retinitis pigmentosa symptoms, includingnyctalopia, constriction of the visual fields, and loss of centralvisual acuity, usually by the second decade of life.
Similarity:
Belongs to the class-II aminoacyl-tRNA synthetasefamily.
Contains 1 WHEP-TRS domain.
Database links:Entrez Gene: 510937 Cow
Entrez Gene: 3035 Human
Entrez Gene: 15115 Mouse
Entrez Gene: 100173931 Orangutan
Entrez Gene: 307492 Rat
Omim: 142810 Human
SwissProt: Q2KI84 Cow
SwissProt: P12081 Human
SwissProt: Q61035 Mouse
SwissProt: Q5R4R2 Orangutan
Unigene: 528050 Human
Unigene: 10528 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.