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Syndrome complex of bone marrow failure and pulmonary fibrosis predicts germline defects in telomerase

Mutations in the essential telomerase components hTERT and hTR cause dyskeratosis congenita, a bone marrow failure syndrome characterized by mucocutaneous features. Some (∼ 3%) sporadic aplastic anemia (AA) and idiopathic pulmonary fibrosis cases also carry mutations in hTERT and hTR. Even though it...

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Autori principali: Parry, Erin M., Alder, Jonathan K., Qi, Xiaodong, Chen, Julian J.-L., Armanios, Mary
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society of Hematology 2011
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3110022/
https://ncbi.nlm.nih.gov/pubmed/21436073
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2010-11-322149
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