Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22.
Disinhibition-dementia-parkinsonism-amyotrophy complex (DDPAC) is defined by familial adult-onset behavioral disturbance, followed by frontal lobe dementia, parkinsonism, and amyotrophy in variable proportions. A genetic etiology of DDPAC was suspected because of the familial clustering in family Mo, despite their wide geographic distribution. We have mapped the DDPAC locus to a 12-cM (sex averaged) region between D17S800 and D17S787 on chromosome 17q21-22. The basis for the variability of the clinical findings and pathology in DDPAC is unknown but suggests that the DDPAC locus should be screened as a candidate locus in family studies of conditions with behavioral abnormalities and neurological degeneration.
Wilhelmsen
K C
KC
Department of Neurology, Columbia-Presbyterian Medical Center, New York, NY 10032.
Lynch
T
T
Pavlou
E
E
Higgins
M
M
Nygaard
T G
TG
D002874:Chromosome Mapping; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics*; D003704:Dementia / Q000209:etiology / Q000235:genetics*; D005260:Female; D005819:Genetic Markers; D005838:Genotype; D006801:Humans; D008126:Lod Score; D008297:Male; D009135:Muscular Diseases / Q000209:etiology / Q000235:genetics*; D009468:Neuromuscular Diseases / Q000235:genetics; D010300:Parkinson Disease / Q000209:etiology / Q000235:genetics*; D010375:Pedigree
D005819:Genetic Markers