Anti-CIRH1A monoclonal antibody (DCABH-11023) Made to order

Rabbit anti-Human CIRH1A monoclonal antibody for WB, ELISA

View other CIRH1A antibodies

Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Species Reactivity
Human
Immunogen
A synthetic peptide of human CIRH1A is used for rabbit immunization.
Conjugate
Unconjugated

Target


Alternative Names
CIRH1A; cirrhosis, autosomal recessive 1A (cirhin); cirhin; CIRHIN; FLJ14728; KIAA1988
Entrez Gene ID
UniProt ID

Product Background


Gene summary
UTP4 (UTP4, Small Subunit Processome Component) is a Protein Coding gene. Diseases associated with UTP4 include cirrhosis, north american indian childhood type and hereditary north american indian childhood cirrhosis. Among its related pathways are Gene Expression and Ribosome biogenesis in eukaryotes. This gene encodes a WD40-repeat-containing protein that is localized to the nucleolus. Mutation of this gene causes North American Indian childhood cirrhosis, a severe intrahepatic cholestasis that results in transient neonatal jaundice, and progresses to periportal fibrosis and cirrhosis in childhood and adolescence. Alternative splicing results in multiple transcript variants.
Antigen Description
This gene encodes a WD40-repeat-containing protein that is localized to the nucleolus. Mutation of this gene causes North American Indian childhood cirrhosis, a severe intrahepatic cholestasis that results in transient neonatal jaundice, and progresses to periportal fibrosis and cirrhosis in childhood and adolescence. 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. Cirhin is a protein that in humans is encoded by the CIRH1A gene. It has been associated with North American Indian childhood cirrhosis (not to be confused with Indian Childhood Cirrhosis which has greatly decreased over the past 100 years and was thought to be secondary to the use of various herbal remedies), a form of cirrhosis of the liver occurring in American Indian children from the Abitibi region of northern Quebec. 0The function about CIRH1A antigen include molecular_function.
Pathway
Ribosome biogenesis in eukaryotes, organism-specific biosystem; Ribosome biogenesis in eukaryotes, conserved biosystem.

Citations


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We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

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References


Carlton, VEH; Pawlikowska, L; et al. Molecular basis of intrahepatic cholestasis. ANNALS OF MEDICINE 36:606-617(2004).
Wilkins, BJ; Lorent, K; et al. p53-Mediated Biliary Defects Caused by Knockdown of cirh1a, the Zebrafish Homolog of the Gene Responsible for North American Indian Childhood Cirrhosis. PLOS ONE 8:-(2013).

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