BD-PT7285

CIR1A Rabbit Polyclonal Antibody

常规抗体

CIRH1A KIAA1988

CIR1A

WB

Human,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

84916

https://www.uniprot.org/uniprot/84916

Q969X6

https://www.uniprot.org/uniprotkb/Q969X6/entry

21771

https://www.uniprot.org/uniprot/21771

Q8R2N2

https://www.uniprot.org/uniprotkb/Q8R2N2

Synthesized peptide derived from human CIR1A AA range: 313-363

This antibody detects endogenous levels of CIR1A at Human/Mouse

WB 1：500-2000

75kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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. [provided by RefSeq, Jan 2016],

Nucleus, nucleolus . Chromosome . Found predominantly at the fibrillar center. .

disease:Defects in CIRH1A are the cause of North American Indian childhood cirrhosis (NAIC) [MIM:604901]. 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.,similarity:Contains 11 WD repeats.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.