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Clinical Features, Genetics and Potential Therapeutic Approaches for Birt-Hogg-Dubé Syndrome

INTRODUCTION: Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder that predisposes to fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax and renal neoplasia. BHD is characterized by germline mutations in tumor suppressor FLCN. Inactivation of the remaining FLCN allele in kidney...

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Publicado en:Expert Opin Orphan Drugs
Autores principales: Schmidt, Laura S., Linehan, W. Marston
Formato: Artigo
Lenguaje:Inglês
Publicado: 2014
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC4646088/
https://ncbi.nlm.nih.gov/pubmed/26581862
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1517/21678707.2014.987124
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