Impaired telomere maintenance and decreased canonical WNT signaling but normal ribosome biogenesis in induced pluripotent stem cells from X-linked dyskeratosis congenita patients

PLoS ONE

Volumn 10, Issue 5, 2015, Pages

  Gu, Bai Wei ^a   Apicella, Marisa ^a   Mills, Jason ^a   Fan, Jian Meng ^a   Reeves, Dara A ^a   French, Deborah ^a   Podsakoff, Gregory M ^a   Bessler, Monica ^a   Mason, Philip J ^a  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DYSKERIN; FRIZZLED PROTEIN; PSEUDOURIDINE; TELOMERASE; WNT PROTEIN; CELL CYCLE PROTEIN; DKC1 PROTEIN, HUMAN; NUCLEAR PROTEIN;

ADOLESCENT; ADULT; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BIOGENESIS; CELL LABELING; CONTROLLED STUDY; DKC1 GENE; DYSKERATOSIS CONGENITA; EMBRYO; ENZYME ACTIVITY; FEMALE; GENE EXPRESSION; GENE LOCUS; GENE MUTATION; HUMAN; HUMAN CELL; MALE; MIDDLE AGED; MOLECULAR PATHOLOGY; MOUSE; NONHUMAN; PHENOTYPE; PLURIPOTENT STEM CELL; PROTEIN EXPRESSION; RIBOSOME; RNA PROCESSING; RNA TRANSLATION; TELOMERE HOMEOSTASIS; TELOMERE SHORTENING; WNT SIGNALING PATHWAY; X CHROMOSOME LINKED DISORDER; X LINKED DYSKERATOSIS CONGENITA; YOUNG ADULT; ANIMAL; CELL CULTURE; GENE EXPRESSION REGULATION; GENETICS; INDUCED PLURIPOTENT STEM CELL; METABOLISM; MUTATION; PATHOLOGY; TELOMERE;

DANIO RERIO; IPS;

ANIMALS; CELL CYCLE PROTEINS; CELLS, CULTURED; DYSKERATOSIS CONGENITA; FEMALE; GENE EXPRESSION REGULATION; HUMANS; INDUCED PLURIPOTENT STEM CELLS; MALE; MICE; MUTATION; NUCLEAR PROTEINS; RIBOSOMES; TELOMERASE; TELOMERE; WNT SIGNALING PATHWAY;

EID: 84930646932     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0127414     Document Type: Article

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