FITC标记的髓鞘缺陷相关蛋白抗体
产品名称： FITC标记的髓鞘缺陷相关蛋白抗体
英文名称： Anti-FAM126A/FITC
产品编号： HZ-11554R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
上海沪震实业有限公司
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
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Rabbit Anti-FAM126A/FITC Conjugated antibody
FITC标记的髓鞘缺陷相关蛋白抗体
| 产品编号 | bs-11554R-FITC |
| 英文名称 | Anti-FAM126A/FITC |
| 中文名称 | FITC标记的髓鞘缺陷相关蛋白抗体 |
| 别 名 | Hyccin; Down regulated by Ctnnb1 a; Down regulated by CTNNB1 protein A; Down-regulated by CTNNB1 protein A; DRCTNNB1A; FAM126A; Family with sequence similarity 126 member A; HCC; HLD5; HYCC1; HYCCI_HUMAN antibody Hyccin; Protein FAM126A. |
| 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 发育生物学 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, |
| 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 | 58kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Hyccin (1-100aa) |
| 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: Hyccin is a 521 amino acid cytoplasmic protein that is widely expressed with highest levels found in heart, brain, placenta, spleen and testis. Belonging to the FAM126 family, hyccin may play a role in the ∫-catenin/Lef signaling pathway. Hyccin is likely involved in the process of myelination of the central and peripheral nervous system. Defects in the gene encoding hyccin are the cause of leukodystrophy hypomyelinating type 5 (HLD5), which is characterized by congenital cataract, progressive neurologic impairment and diffuse myelin deficiency. Individuals affected by HLD5 experience progressive pyramidal and cerebellar dysfunction along with muscle weakness in the lower limbs. Hyccin exists as two alternatively spliced isoforms and is encoded by a gene located on human chromosome 7. Function: May have a role in the beta-catenin/Lef signaling pathway. May have a role in the process of myelination of the central and peripheral nervous system. Subcellular Location: Cytoplasm. Membrane. According to PubMed:10910037, it is mainly cytoplasmic while according to PubMed:16951682, it is a membrane protein. Tissue Specificity: Widely expressed. Highest levels in heart, brain, placenta, spleen and testis. DISEASE: Defects in FAM126A are the cause of leukodystrophy hypomyelinating type 5 (HLD5) [MIM:610532]. This disorder is characterized by congenital cataract, progressive neurologic impairment, and diffuse myelin deficiency. Affected individuals experience progressive pyramidal and cerebellar dysfunction, muscle weakness and wasting prevailingly in the lower limbs. Mental deficiency ranges from mild to moderate. Similarity: Belongs to the FAM126 family. Database links: Entrez Gene: 84668 Human Entrez Gene: 84652 Mouse Omim: 610531 Human SwissProt: Q9BYI3 Human SwissProt: Q6P9N1 Mouse Unigene: 85603 Human Unigene: 304976 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
Hyccin是一种广泛存在于心脏、脑、胎盘、脾脏和睾丸中的521氨基酸胞质蛋白。HycCin在FAM126家族中可能在β-catenin／LeF信号通路中发挥作用。Hyccin可能参与中枢和外周神经系统髓鞘形成的过程。编码HycCin基因的缺陷是导致脑白质营养不良的5型（HLD5），其特征是先天性白内障、进行性神经损害和弥漫性髓磷脂缺乏。受HLD5影响的个体经历渐进性锥体和小脑功能障碍以及下肢肌肉无力。HycCin作为两个交替剪接的亚型存在，并由位于人类染色体7上的基因编码。