Mouse Genome Informatics
hm
    Als2tm1Cai/Als2tm1Cai
involves: 129X1/SvJ * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• 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 Ccstm1Pcw homozygotes

growth/size/body
• 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
• 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
• 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
• 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
• 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
• 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 Ccstm1Pcw homozygotes
• increased sensitivity to paraquat-induced in vitro neuronal cell death is seen in homozygous mice compared to wild-type littermates

nervous system
N
• no significant changes in peripheral nerve conduction or electromyography and no gross neuropathological abnormalities are seen (J:100336)

Mouse Models of Human Disease
OMIM IDRef(s)
NOT Amyotrophic Lateral Sclerosis 2, Juvenile; ALS2 205100 J:100336