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Genetic linkage of vitelliform macular degeneration (Best's disease) to chromosome 11q13
Journal article   Peer reviewed

Genetic linkage of vitelliform macular degeneration (Best's disease) to chromosome 11q13

Edwin M Stone, Brian E Nichols, Luan M Streb, Alan E Kimura and Val C Sheffield
Nature genetics, Vol.1(4), pp.246-250
07/1992
DOI: 10.1038/ng0792-246
PMID: 1302019

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Abstract

Macular degeneration is the most common cause of legal blindness in older patients in developed countries. Best's vitelliform dystrophy is an early-onset, autosomal dominant form of macular degeneration characterized by an egg-yolk-like collection of lipofuscin beneath the pigment epithelium of the retinal macula. Fifty-seven members of a five-generation family affected with this disease were studied. A combination of ophthalmoscopy and electro-oculography was used for diagnosis; 29 patients were found to be affected and 16 unaffected. Linkage analysis mapped the disease-causing gene to chromosome 11q13. Three markers in this region were found to be significantly linked (Zmax > 3.0) to the disease. Multipoint analysis yielded a maximum Lod score of 9.3 in the interval between markers INT2 and D11S871.
Probability Macular Degeneration - physiopathology Humans Genotype Male Chromosome Mapping Visual Acuity Macular Degeneration - genetics Pedigree Female Chromosomes, Human, Pair 11 Odds Ratio Fluorescein Angiography Genetic Linkage Macular Degeneration - pathology

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