نتایج جستجو برای: leber hereditomy optic neurophaty

تعداد نتایج: 46273  

Journal: :Vision Research 1997
Neil Howell

The predominant manifestation of Leber hereditary optic neuropathy (LHON) is a sudden and usually severe bilateral loss of central vision, most often in the mid-20s, that is due to a degeneration of the ganglion cell layer and optic nerve. LHON is an inherited form of blindness in which a mutation in the mitochondrial genome (mtDNA) is the primary etiological event. More than 95% of the LHON pe...

2017
Chunyan Liao Neil Ashley Alan Diot Karl Morten Kanchan Phadwal Andrew Williams Ian Fearnley Lyndon Rosser Jo Lowndes Carl Fratter David J.P. Ferguson Laura Vay Gerardine Quaghebeur Isabella Moroni Stefania Bianchi Costanza Lamperti Susan M. Downes Kamil S. Sitarz Padraig J. Flannery Janet Carver Eszter Dombi Daniel East Matilde Laura Mary M. Reilly Heather Mortiboys Remko Prevo Michelangelo Campanella Matthew J. Daniels Massimo Zeviani Patrick Yu-Wai-Man Anna Katharina Simon Marcela Votruba Joanna Poulton

OBJECTIVE To investigate mitophagy in 5 patients with severe dominantly inherited optic atrophy (DOA), caused by depletion of OPA1 (a protein that is essential for mitochondrial fusion), compared with healthy controls. METHODS Patients with severe DOA (DOA plus) had peripheral neuropathy, cognitive regression, and epilepsy in addition to loss of vision. We quantified mitophagy in dermal fibro...

Journal: :Investigative ophthalmology & visual science 2007
Khaled K Abu-Amero Jose Morales Mazen N Osman Thomas M Bosley

PURPOSE Certain types of glaucoma are linked to nuclear genetic mutations or to mitochondrial disturbances. In this study, patients with primary angle-closure glaucoma (PACG) were examined for mutations in nuclear genes reported to be associated with glaucoma and for possible mitochondrial abnormalities. METHODS In patients with PACG, the nuclear genes MYOC, OPTN, CYP1B1, WDR36, OPA1, and OPA...

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