Phenotypes Associated with This Genotype

Genotype
MGI:3589591

• Allelic Composition: Als2^tm1Cai/Als2^tm1Cai
• Genetic Background: involves: 129X1/SvJ * C57BL/6J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

increased susceptibility to xenobiotic induced morbidity/mortality ( J:100336 )

• intraperitoneal injection of 35 mg/kg paraquat induced death in about 50% of homozygotes compared to 0% of control mice

• homozygotes are not as sensitive to paraquat-induced mortality as are Ccs^tm1Pcw homozygotes

growth/size/body

increased body weight ( J:100336 )

• body weight is normal in young mice but increased by 18% and 25% at 12 and 20 months of age, respectively, compared to wild-type littermates

behavior/neurological

abnormal motor learning ( J:100336 )

• no significant change in the latency to fall from a rotating rod is seen between 9 and 20 months of age and in repeated trials over multiple days homozygotes do not perform better in the first trials of day compared to their performance in the last trial of the preceding day

decreased exploration in new environment ( J:100336 )

• a decrease in locomotor activity is seen in a novel environment; however, gait, grip, muscle tone, and startle response are all normal and no decrease in spontaneous activity is seen in a Flex-Field apparatus

increased anxiety-related response ( J:100336 )

• homozygotes spend less time in the center of an open field and spend less time and make fewer visits to the open arms of an elevated plus maze

impaired coordination ( J:100336 )

• at 12 and 20 months of age homozygotes display a decreased latency to fall from a rotating rod compared to wild-type littermates

homeostasis/metabolism

increased susceptibility to xenobiotic induced morbidity/mortality ( J:100336 )

• intraperitoneal injection of 35 mg/kg paraquat induced death in about 50% of homozygotes compared to 0% of control mice

• homozygotes are not as sensitive to paraquat-induced mortality as are Ccs^tm1Pcw homozygotes

increased physiological sensitivity to xenobiotic ( J:100336 )

• increased sensitivity to paraquat-induced in vitro neuronal cell death is seen in homozygous mice compared to wild-type littermates

nervous system

nervous system phenotype ( J:100336 )

N • no significant changes in peripheral nerve conduction or electromyography and no gross neuropathological abnormalities are seen

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
NOT	amyotrophic lateral sclerosis type 2	DOID:0060194	OMIM:205100	J:100336