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- bs-13958P
- 2025年10月16日
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- 规格：
500ug
| 产品编号 | bs-13958P |
| 英文名称 | Cirhin Antibody Blocking Peptide |
| 中文名称 | 常染色体隐性遗传肝硬化1A封闭多肽 |
| 英文别名 | CIRH1A; CIRH 1A; Cirhin; Cirrhosis, autosomal recessive 1A (cirhin); FLJ17146; KIAA1988; NAIC; testis expressed gene 292; TEX292; CIR1A_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Epigenetics and Nuclear Signaling > Nuclear Signaling Pathways > NFkB pathway Signal Transduction > Signaling Pathway > Nuclear Signaling > NFkB Pathway |
| 亚基 | Interacts with HIVEP1. Forms a complex with NOL11, UTP15, WDR43 and WDR75; within this complex, directly interacts with NOL11. |
| 亚细胞定位 | Nucleus; nucleolus |
| 相似性 | Contains 11 WD repeats. |
| 功能 | 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. |
| 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 | 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. |
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Cirhin Antibody Blocking Peptide(bs-13958P)-500ug
￥880
