Early detection of mouse wobbler mutation: a model of pathological motoneurone death.
The mouse recessive mutation wobbler, carried by the C57BL/6J strain, is a naturally occurring model of motoneurone death. The gene is unknown and in the absence of predictive markers, mutants have to be diagnosed by phenotypic criteria at 4 weeks after birth. We localized the wobbler gene to chromosome 11 at 0.98 +/- 1.1 cM from the glutamine synthetase (Glns) gene. A polymorphic allele of the Glns gene was then introduced into the congenic wobbler strain by intraspecific crossing. One-quarter of the offspring expressed the same phenotypic mutation as true wobbler and were detectable by PCR, as they are homozygous for the wobbler-linked Glns allele. The new mutants exhibit motoneurone degeneration despite the new genetic background.
INSERM U 153, Paris, France.
V
V
des Portes
M
M
Coulpier
J
J
Melki
P A
PA
Dreyfus
D000818:Animals; D001483:Base Sequence; D016923:Cell Death / Q000502:physiology; D002874:Chromosome Mapping; D004195:Disease Models, Animal; D008040:Genetic Linkage; D006720:Homozygote; D006801:Humans; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D008969:Molecular Sequence Data; D009046:Motor Neurons / Q000473:pathology*; D015345:Oligonucleotide Probes; D016133:Polymerase Chain Reaction; D012150:Polymorphism, Restriction Fragment Length; D013116:Spinal Cord / Q000473:pathology
D015345:Oligonucleotide Probes