Faculty Research 1990 - 1999

Title

Mapping of the motor neuron degeneration (Mnd) gne, a mouse model of amyotrophic lateral sclerosis (ALS).

Document Type

Article

Publication Date

1992

Keywords

Amyotrophic-Lateral-Sclerosis: ge, Animal, Base-Sequence, Chromosome-Mapping, Crosses-Genetic, Disease-Models-Animal, DNA-Mutational-Analysis, Genetic-Markers, Mice, Mice-Mutant-Strains, Molecular-Sequence-Data, Motor-Neuron-Disease: ge, Nerve-Degeneration: ge, Proviruses: ge, SUPPORT-NON-U-S-GOVT, SUPPORT-U-S-GOVT-P-H-S

JAX Source

Genomics 1992 Jul;13(3):797-802

Grant

R35CA44385/CA/NCI

Abstract

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.

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