Telomere elongation in induced pluripotent stem cells from dyskeratosis congenita patients
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چکیده
منابع مشابه
Impaired Telomere Maintenance and Decreased Canonical WNT Signaling but Normal Ribosome Biogenesis in Induced Pluripotent Stem Cells from X-Linked Dyskeratosis Congenita Patients
Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome characterized by the presence of short telomeres at presentation. Mutations in ten different genes, whose products are involved in the telomere maintenance pathway, have been shown to cause DC. The X-linked form is the most common form of the disease and is caused by mutations in the gene DKC1, encoding the protein dyskeri...
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Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome characterized by cutaneous symptoms, including hyperpigmentation and nail dystrophy. Some forms of DC are caused by mutations in telomerase, the enzyme that counteracts telomere shortening, suggesting a telomere-based disease mechanism. However, mice with extensively shortened telomeres due to telomerase deficiency do not ...
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1 Supplementary Materials Bone marrow skeletal stem/progenitor cell defects in patients with dyskeratosis congenita and telomere biology disorders Arun Balakumaran, Prasun J. Mishra, Edyta Pawelczyk, Sayuri Yoshizawa, Brian Sworder, Natasha Cherman, Sergei A. Kuznetsov, Paolo Bianco, Neelam Giri, Sharon A. Savage, Glenn Merlino, Bogdan Dumitriu, Cynthia E. Dunbar, Neal S. Young, Blanche P. Alte...
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Regenerative capacity of mammals is limited and can rarely regenerate a specific organ or tissue fully. Due to these limitations, regenerative medicine seeks efficient and safe cell sources for regeneration of damaged tissues and organs or treatment for incurable diseases. Human embryonic stem cells (HESCs) hold two important properties called self renewal and pluripotency. However, the use of ...
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1Department of Pediatrics, Division of Hematology/Oncology, University of Iowa Children’s Hospital, Iowa City; 2Terry Fox Laboratory, British Columbia Cancer Agency, Vancouver, BC; 3Department of Microbiology, University of Iowa, Iowa City; 4Department of Pathology, University of Iowa, Iowa City; 5Department of Medical Genetics, University of British Columbia, Vancouver, BC; and 6Division of He...
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ژورنال
عنوان ژورنال: Nature
سال: 2010
ISSN: 0028-0836,1476-4687
DOI: 10.1038/nature08792