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Clinical variability in maternally inherited leber...
Journal article

Clinical variability in maternally inherited leber hereditary optic neuropathy with the G14459A mutation

Abstract

Spasticity and dystonia have been associated with mitochondrial (mt) DNA mutations at A11696G, G14459A, and T14596A. We describe the clinical features and molecular analysis of two Caucasian pedigrees with the 14,459 guanosine (G) --> adenine (A) transition. The maternally inherited Leber hereditary optic neuropathy (LHON) phenotypes showed extreme clinical variability and the only screening test that was abnormal in the patient with …

Authors

Tarnopolsky MA; Baker SK; Myint T; Maxner CE; Robitaille J; Robinson BH

Journal

American Journal of Medical Genetics Part A, Vol. 124A, No. 4, pp. 372–376

Publisher

Wiley

Publication Date

February 2004

DOI

10.1002/ajmg.a.20449

ISSN

1552-4825