Product Name
CIRH1A, Monoclonal Antibody
Full Product Name
Mouse monoclonal antibody Anti-Human CIRH1A
Product Synonym Names
Homo sapiens cirrhosis, autosomal recessive 1A (cirhin) (CIRH1A), mRNA.; CIRHIN; FLJ14728; KIAA1988; NAIC; TEX292
Product Gene Name
anti-CIRH1A antibody
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Concentration
100 ug/ml (1.0 ml) (lot specific)
Preparation
This antibody was purified using protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (approximately 95%) fetal bovine serum.
Sterility
Filtered through a 0.22 um membrane.
Other Notes
Small volumes of anti-CIRH1A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-CIRH1A antibody
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. [provided by RefSeq][NCBI Entrez Gene Summary]
Applications Tested/Suitable for anti-CIRH1A antibody
Dot Blot (DB), Immunocytochemistry (ICC)
Quality Control of anti-CIRH1A antibody
Western blot analysis of immunized recombinant protein, using anti-CIRH1A monoclonal antibody.
Quality Control #2 of anti-CIRH1A antibody
Arrow indicates the region of immunized recombinant protein carrying 50-200 amino acids.
NCBI/Uniprot data below describe general gene information for CIRH1A. It may not necessarily be applicable to this product.
NCBI Accession #
NP_116219.2
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NCBI GenBank Nucleotide #
NM_032830
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UniProt Secondary Accession #
Q8NCD9; Q8TF14; Q96SP0; Q96SR9; Q96SZ9; Q96T13; Q9BWK6[Other Products]
UniProt Related Accession #
Q969X6[Other Products]
Molecular Weight
69,639 Da
NCBI Official Full Name
Homo sapiens cirrhosis, autosomal recessive 1A (cirhin) (CIRH1A), mRNA
NCBI Official Synonym Full Names
cirrhosis, autosomal recessive 1A (cirhin)
NCBI Official Symbol
CIRH1A??[Similar Products]
NCBI Official Synonym Symbols
NAIC; UTP4; CIRHIN; TEX292
??[Similar Products]
NCBI Protein Information
cirhin; UTP4, small subunit (SSU) processome component, homolog; testis expressed gene 292
UniProt Protein Name
Cirhin
UniProt Gene Name
CIRH1A??[Similar Products]
UniProt Entry Name
CIR1A_HUMAN
NCBI Summary for CIRH1A
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. [provided by RefSeq, Jul 2008]
UniProt Comments for CIRH1A
CIRH1A: May be a transcriptional regulator. Acts as a positive regulator of HIVEP1 which specifically binds to the DNA sequence 5'-GGGACTTTCC-3' found in enhancer elements of numerous viral promoters such as those of HIV-1, SV40, or CMV. Defects in CIRH1A are the cause of North American Indian childhood cirrhosis (NAIC). 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. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Unknown function; Nucleolus
Chromosomal Location of Human Ortholog: 16q22.1
Cellular Component: nucleolus
Molecular Function: protein binding
Biological Process: transcription, DNA-dependent; regulation of transcription, DNA-dependent; maturation of SSU-rRNA
Disease: North American Indian Childhood Cirrhosis
Research Articles on CIRH1A
1. Cirhin is a transcriptional regulatory factor of this NF-kappaB sequence and could be a participant in the regulation of other genes with NF-kappaB responsive elements.
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