نتایج جستجو برای: pelizaeus

تعداد نتایج: 366  

Journal: :Brain : a journal of neurology 2005
Nicole I Wolf Erik A Sistermans Maria Cundall Grace M Hobson Angelique P Davis-Williams Rodger Palmer Paula Stubbs Sally Davies Milda Endziniene Yvonne Wu Wui K Chong Sue Malcolm Robert Surtees James Y Garbern Karen J Woodward

We describe five boys from different families with an atypically severe form of Pelizaeus-Merzbacher disease (PMD) who have three, and in one case, five copies of the proteolipid protein (PLP1) gene. This is the first report of more than two copies of PLP1 in PMD patients and clearly demonstrates that severe clinical symptoms are associated with increased PLP1 gene dosage. Previously, duplicati...

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