FITC标记的常染色体隐性遗传肝硬化1A抗体
产品名称： FITC标记的常染色体隐性遗传肝硬化1A抗体
英文名称： Anti-Cirhin/FITC
产品编号： HZ-13958R-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
- 电话 : 139****0749 点击查看
- 传真 : 点击查看
- 邮箱 : www.shzbio.net
- 二维码 : 点击查看
Rabbit Anti-Cirhin/FITC Conjugated antibody
FITC标记的常染色体隐性遗传肝硬化1A抗体
| 英文名称 | Anti-Cirhin/FITC |
| 中文名称 | FITC标记的常染色体隐性遗传肝硬化1A抗体 |
| 别 名 | CIRH1A; CIRH 1A; Cirhin; Cirrhosis, autosomal recessive 1A (cirhin); FLJ17146; KIAA1988; NAIC; testis expressed gene 292; TEX292; CIR1A_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 染色质和核信号 信号转导 转录调节因子 细菌及病毒 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Rat, Dog, Horse, |
| 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 | 77kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Cirhin |
| 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: CIRH1A (cirrhosis, autosomal recessive 1A), also designated cirhin, NAIC or TEX292, is a 686 amino acid protein that, when mutated, causes a severe autosomal recessive intrahepatic cholestasis known as North American Indian childhood cirrhosis (NAIC). NAIC is found in aboriginal children from northwestern Quebec, and is characterized by transient neonatal jaundice which progresses to biliary cirrhosis, portal hypertension and periportal fibrosis during childhood and adolescence. Localizing to nucleolus, CIRH1A contains eleven WD repeats, exists as three alternatively spliced isoforms and is encoded by a gene that maps to human chromosome 16q22.1. Chromosome 16 encodes over 900 genes in approximately 90 million base pairs, makes up nearly 3% of human cellular DNA and is associated with a variety of genetic disorders. Function: Defects in Cirhin are the cause of North American Indian childhood cirrhosis. NAIC is a severe autosomal recessive intrahepatic cholestasis, originally described in Ojibway-Cree children from northwestern Quebec. NAIC typically presents with transient neonatal jaundice, in a child who is otherwise healthy, and progresses to biliary cirrhosis and portal hypertension. Biochemical and histopathological features suggest involvement of the bile ducts rather than of the bile canaliculi. They include elevated gamma glutamyltransferase and alkaline phosphatase levels, and, typically, marked fibrosis around bile ducts. Clinically, NAIC is distinct from other nonsyndromic familial cholestases because of its marked cholangiopathic features and severe degree of fibrosis on liver histology. Subunit: Interacts with HIVEP1. Forms a complex with NOL11, UTP15, WDR43 and WDR75; within this complex, directly interacts with NOL11. Subcellular Location: Nucleus; nucleolus DISEASE: North American Indian childhood cirrhosis (NAIC) [MIM:604901]: Severe autosomal recessive intrahepatic cholestasis, originally described in Ojibway-Cree children from northwestern Quebec. NAIC typically presents with transient neonatal jaundice, in a child who is otherwise healthy, and progresses to biliary cirrhosis and portal hypertension. Biochemical and histopathological features suggest involvement of the bile ducts rather than of the bile canaliculi. They include elevated gamma glutamyltransferase and alkaline phosphatase levels, and, typically, marked fibrosis around bile ducts. Clinically, NAIC is distinct from other nonsyndromic familial cholestases because of its marked cholangiopathic features and severe degree of fibrosis on liver histology. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Contains 11 WD repeats. Database links: Entrez Gene: 84916 Human Omim: 607456 Human SwissProt: Q969X6 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
CIRH1A（肝硬化，常染色体隐性1A），也称为肝素、NAIC或TEX292，是一种686个氨基酸的蛋白质，当发生突变时，会引起严重的常染色体隐性肝内胆汁淤积症，称为北美印第安儿童期肝硬化（NAIC）。NAIC在魁北克西北部的土著儿童中发现，其特点是短暂的新生儿黄疸，在儿童期和青春期发展为胆汁性肝硬化、门脉高压和门脉周围纤维化。CIRH1A定位于核仁，包含11个WD重复，作为三种可选择的剪接异构体存在，并且由映射到人类染色体16q22.1的基因编码。染色体16在大约9千万碱基对中编码900多个基因，占人类细胞DNA的近3％，并且与多种遗传病有关。