Reduced fecundity in male ALS gene-carriers.
In order to study genetic aspects in multicase families, 89 amyotrophic lateral sclerosis (ALS) and 214 Parkinson disease (PD) kindreds were analyzed in parallel studies. Obligate gene-carriers were identified as described previously [Chatkupt et al., 1992: Am J Med Genet 44:508-512]. There were fewer children per gene-carrier male (2.42) than per gene-carrier female (3.25, Student's t-test, P < .0003) for ALS but not for other diseases. The data taken together suggest that fecundity in ALS gene-carriers was reduced in males, possibly as a result of reduced fertility. Since childbearing is usually accomplished well before the onset of neurological symptoms in ALS, and since reduced fecundity was found in male ALS gene-carriers, these findings raise the possibility that an ALS gene might have a pleiotrophic effect on fertility in males occurring decades before the onset of neurological symptoms. Evidence is presented linking reactive oxygen species to reduced fertility in males. Alternatively, decreased or nonfunctional androgen receptors could play a role.
Johnson
W G
WG
Department of Neurology, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School, New Brunswick, USA.
Lucek
P R
PR
Chatkupt
S
S
Furman
Y
Y
Lustenberger
A
A
Lazzarini
A
A
D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology / Q000235:genetics* / Q000503:physiopathology; D005260:Female; D005298:Fertility / Q000235:genetics*; D005799:Genes, Dominant; D006579:Heterozygote; D006801:Humans; D008297:Male; D010300:Parkinson Disease / Q000235:genetics / Q000503:physiopathology; D010375:Pedigree; D017354:Point Mutation; D012727:Sex Characteristics; D013482:Superoxide Dismutase / Q000235:genetics
D013482:Superoxide Dismutase