- ￥880
- Bioss
- bs-20281P
- 2025年10月16日
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500ug
| 产品编号 | bs-20281P |
| 英文名称 | HARS Antibody Blocking Peptide |
| 中文名称 | 组氨酸tRNA连接酶封闭多肽 |
| 英文别名 | 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. |
| 纯化方法 | HPLC |
| 研究领域 | Epigenetics and Nuclear Signaling > DNA / RNA > Translation > tRNA synthetase Immunology > Immune System Diseases > Autoimmune |
| 亚细胞定位 | Cytoplasmic |
| 组织特异性 | Brain, heart, liver and kidney. |
| 翻译后修饰 | 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. |
| 相似性 | Belongs to the class-II aminoacyl-tRNA synthetasefamily. Contains 1 WHEP-TRS domain. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
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HARS Antibody Blocking Peptide(bs-20281P)-500ug
￥880
