Mapping of the motor neuron degeneration (Mnd) gene, a mouse model of amyotrophic lateral sclerosis (ALS).
The motor neuron degeneration mutation (Mnd) causes a late-onset, progressive degeneration of upper and lower motor neurons in mice. After establishing genetic and environmental conditions that distinguish the phenotypes of Mnd/Mnd from +/Mnd mice, Mnd was mapped to proximal Chr 8, using endogenous retroviruses as markers. The map location was confirmed with additional linked polymorphic markers. The outcross/intercross matings to the strain AKR/J, which were used to follow the segregation of the retroviral markers with respect to Mnd, also revealed the existence of a timing effect. Approximately one-fourth of the affected Mnd/Mnd F2 progeny showed accelerated disease. The Mnd mouse model should allow study of mechanisms affecting onset and progression of specific neuronal degeneration in both animal and human neurological disease.
Messer
A
A
Wadsworth Center for Laboratories and Research, New York State Department of Health.
Plummer
J
J
Maskin
P
P
Coffin
J M
JM
Frankel
W N
WN
D000367:Age Factors; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics; D000818:Animals; D001483:Base Sequence; D002874:Chromosome Mapping; D003433:Crosses, Genetic; D004252:DNA Mutational Analysis; D004195:Disease Models, Animal; D005819:Genetic Markers; D051379:Mice; D008817:Mice, Mutant Strains; D008969:Molecular Sequence Data; D016472:Motor Neuron Disease / Q000235:genetics*; D009410:Nerve Degeneration / Q000235:genetics*; D011533:Proviruses / Q000235:genetics
D005819:Genetic Markers