p53-mediated biliary defects caused by knockdown of cirh1a, the zebrafish homolog of the gene responsible for North American Indian Childhood Cirrhosis

North American Indian Childhood Cirrhosis (NAIC) is a rare, autosomal recessive, progressive cholestatic disease of infancy affecting the Cree-Ojibway first Nations of Quebec. All NAIC patients are homozygous for a missense mutation (R565W) in CIRH1A, the human homolog of the yeast nucleolar protein...

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Veröffentlicht in:	PloS one 2013-10, Vol.8 (10), p.e77670-e77670
Hauptverfasser:	Wilkins, Benjamin J, Lorent, Kristin, Matthews, Randolph P, Pack, Michael
Format:	Artikel
Sprache:	eng
Schlagworte:	Analysis Anemia Animal models Animals Apoptosis Bile Biopsy Biosynthesis Birth defects Cell cycle Cellular stress response Childhood Children Cirrhosis Congenital defects Congenital diseases Cytology Danio rerio Defects Disease Embryos Epithelial cells Evolution Fluorescence Gallbladder diseases Gene expression Genetic aspects Genetic disorders Hepatocytes Hepatocytes - metabolism Homology Humans Hypertension In Situ Hybridization Larva - metabolism Larvae Liver Liver - cytology Liver - metabolism Liver cirrhosis Liver Cirrhosis, Biliary - genetics Liver Cirrhosis, Biliary - metabolism Medicine Metabolism Missense mutation Mutation Mutation, Missense - genetics Native Americans Native North Americans Nucleoli Oligonucleotides p53 Protein Pathogenesis Physiological aspects Proteins Ribonucleic acid Ribonucleoproteins - genetics Ribosomal RNA RNA RNA processing rRNA Transcription Tumor proteins Tumor Suppressor Protein p53 - genetics Tumor Suppressor Protein p53 - metabolism Yeast Yeasts Zebrafish Zebrafish Proteins - genetics Zebrafish Proteins - metabolism
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