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Western Blot (WB) (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.)

Rabbit anti-Human, Mouse CIRH1A Polyclonal Antibody | anti-CIRH1A antibody

CIRH1A Polyclonal Antibody

Gene Names
UTP4; NAIC; CIRH1A; CIRHIN; TEX292
Reactivity
Human, Mouse
Applications
Western Blot
Purity
Affinity purification
Synonyms
CIRH1A; Polyclonal Antibody; CIRH1A Polyclonal Antibody; NAIC; UTP4; CIRHIN; TEX292; anti-CIRH1A antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human, Mouse
Clonality
Polyclonal
Isotype
IgG
Purity/Purification
Affinity purification
Sequence Length
686
Applicable Applications for anti-CIRH1A antibody
Western Blot (WB)
Application Notes
WB: 1:500 - 1:2000
Immunogen
Recombinant protein of human CIRH1A
Preparation and Storage
Store at -20 degree C. Avoid freeze / thaw cycles.

Western Blot (WB)

(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.)

Western Blot (WB) (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.)
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

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
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
NCBI Official Synonym Symbols
NAIC; CIRH1A; CIRHIN; TEX292
NCBI Protein Information
U3 small nucleolar RNA-associated protein 4 homolog
UniProt Protein Name
U3 small nucleolar RNA-associated protein 4 homolog
UniProt Gene Name
UTP4
UniProt Entry Name
UTP4_HUMAN

NCBI 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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]

Uniprot Description

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

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Product Notes

The CIRH1A utp4 (Catalog #AAA9130409) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The CIRH1A Polyclonal Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's CIRH1A can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:500 - 1:2000. Researchers should empirically determine the suitability of the CIRH1A utp4 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "CIRH1A, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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