Product Name
CIRH1A, cDNA Clone
Full Product Name
CIRH1A cDNA Clone
Product Gene Name
CIRH1A cdna clone
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
atgggtgaat ttaaggtcca tcgagtacgt ttctttaatt atgttccatc aggaatccgc tgtgtggctt acaataacca gtcaaacaga ttggctgttt cacgaacaga tggcactgtg gaaatttata acttgtcagc aaactacttt caggagaaat ttttcccagg tcatgagtct cgggctacag aagctttgtg ctgggcagaa ggacagcgac tctttagtgc tgggctcaat ggcgagatta tggagtatga tttacaggcg ttaaacatca agtatgctat ggatgccttt ggaggaccta tttggagcat ggctgccagc cccagtggct ctcaactttt ggttggttgt gaagatggat ctgtgaaact atttcaaatt accccagaca aaatccagtt tgaaagaaat tttgatcggc agaaaagtcg catcctgagt ctcagctggc atccctctgg tacccacatt gcagctggtt ccatagacta cattagtgtg tttgatgtca aatcaggcag cgctgttcat aagatgattg tggacaggca gtatatgggc gtgtctaagc ggaagtgcat cgtgtggggt gtcgccttct tgtccgatgg cactatcata agtgtggact ctgctgggaa ggtgcagttc tgggactcag ccactgggac gcttgtgaag agccatctca tcgctaatgc tgacgtgcag tccattgctg tagctgacca agaagacagt ttcgtggtgg gcacagccga gggaacagtc ttccattttc agctggtccc tgtgacatct aacagcagtg agaagcagtg ggtgcggaca aaaccgttcc agcatcacac tcatgacgtg cgcactgtgg cccacagccc aacagcgctg atatctggag gcactgacac ccacttagtc tttcgtcctc tcatggagaa ggtggaagta aagaattacg atgccgctct ccgaaaaatc acctttcccc accgatgtct catctcctgt tctaaaaaga ggcagcttct cctcttccag tttgctcatc acttagaact ttggcgactg ggatccacag ttgcaacagg caagaatggg gatactcttc cactctctaa aaatgcagat catttactgc acctaaagac aaagggtcct gagaacatta tctgtagctg tatctcccca tgtggaagtt ggatagccta ttctacagtt tctcggtttt ttctctatcg gctgaattat gaacatgaca acataagcct caaaagggtt tccaaaatgc cagcattcct tcgctctgcc cttcagattt tgttttctga agattcaaca aagctctttg tagcatcaaa tcaaggagct ctgcatattg ttcagctgtc aggaggaagc ttcaagcacc tgcatgcttt ccagcctcag tcaggaacag tggaggccat gtgtcttttg gcagtcagtc cagatgggaa ttggctagct gcatcaggta ccagtgctgg agtccatgtc tacaacgtaa aacagctaaa gcttcactgc acggtgcctg cttacaattt cccagtgact gctatggcta ttgcccccaa taccaacaac cttgtcatcg ctcattcgga ccagcaggta tttgagtaca gcatcccaga caaacagtat acagattgga gccggactgt ccagaagcag ggctttcacc acctttggct ccaaagggat actcctatca cacacatcag ttttcatccc aagagaccga tgcacatcct tctccatgat gcctacatgt tctgcatcat tgacaagtca ttgccccttc caaatgacaa aaccttactc tacaatccat ttcctcccac gaatgaatca gatgtcatcc ggaggcgcac agctcatgct tttaaaattt ctaagatata taagcctcta ctcttcatgg atcttttgga tgaaagaaca ctcgtggcag tagaacggcc tctggatgac atcattgctc agctcccacc acccattaaa aagaagaaat ttggaaccta a
Clone Sequence Report
Provided with product shipment
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of CIRH1A cdna clone 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.
NCBI/Uniprot data below describe general gene information for CIRH1A. It may not necessarily be applicable to this product.
NCBI Accession #
BC009348
[Other Products]
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), mRNA
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: chromosome; fibrillar center; nucleolus; nucleoplasm
Molecular Function: protein binding
Biological Process: maturation of SSU-rRNA; regulation of transcription, DNA-dependent; rRNA processing
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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Disclaimer
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