Product Name
CIRH1A, Polyclonal Antibody
Popular Item
Full Product Name
CIRH1A Polyclonal Antibody
Product Synonym Names
NAIC; UTP4; CIRHIN; TEX292
Product Gene Name
anti-CIRH1A antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q969X6
Species Reactivity
Human, Mouse
Purity/Purification
Affinity purification
Immunogen
Recombinant protein of human CIRH1A
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Preparation and Storage
Store at -20 degree C. Avoid freeze / thaw cycles.
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.
Product Categories/Family for anti-CIRH1A antibody
Polyclonal
Applications Tested/Suitable for anti-CIRH1A antibody
Western Blot (WB)
Application Notes for anti-CIRH1A antibody
WB: 1:500 - 1:2000
Western Blot (WB) of anti-CIRH1A antibody
Western blot analysis of extracts of various cell lines, using CIRH1A antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 5s.
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_032830.2
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UniProt Primary Accession #
Q969X6
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UniProt Secondary Accession #
Q8NCD9; Q8TF14; Q96SP0; Q96SR9; Q96SZ9; Q96T13; Q9BWK6[Other Products]
UniProt Related Accession #
Q969X6[Other Products]
Molecular Weight
Calculated MW: 77kDa
Molecular Weight: 686
NCBI Official Full Name
U3 small nucleolar RNA-associated protein 4 homolog isoform 1
NCBI Official Synonym Full Names
UTP4, small subunit processome component
NCBI Official Symbol
UTP4??[Similar Products]
NCBI Official Synonym Symbols
NAIC; CIRH1A; CIRHIN; TEX292
??[Similar Products]
NCBI Protein Information
U3 small nucleolar RNA-associated protein 4 homolog
UniProt Protein Name
U3 small nucleolar RNA-associated protein 4 homolog
UniProt Synonym Protein Names
Cirhin; UTP4 small subunit processome component
UniProt Gene Name
UTP4??[Similar Products]
UniProt Entry Name
UTP4_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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]
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: Nucleolus; Unknown function
Chromosomal Location of Human Ortholog: 16q22.1
Cellular Component: nucleolus
Molecular Function: protein binding
Biological Process: maturation of SSU-rRNA; regulation of transcription, DNA-dependent; transcription, DNA-dependent
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.
Precautions
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