Phenotypes Associated with This Genotype

Genotype
MGI:5484560

• Allelic Composition: Gt(ROSA)26Sor^{tm1.1(CAG-PLS3,-GFP)Bwir}/Gt(ROSA)26Sor⁺; Smn1^tm1Hung/Smn1^tm1Hung; Tg(SMN2)2Hung/0
• Genetic Background: B6N.Cg-Gt(ROSA)26Sor^{tm1.1(CAG-PLS3,-GFP)Bwir} Smn1^tm1Hung Tg(SMN2)2Hung

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

mortality/aging

premature death ( J:193844 )

• as in Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

nervous system

abnormal motor neuron morphology ( J:193844 )

• increased motor neuron size with increased proprioceptive nerves compared with Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

abnormal proprioceptive neuron morphology ( J:193844 )

• increased proprioceptive nerves in contact with motor neurons compared with Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

abnormal neuromuscular synapse morphology ( J:193844 )

• at P4, P8 and P11, endplate size is increased compared to in Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

• at P1, P4 and P8, endplates exhibit an increase in axon input compared with Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:193844 )

• as in Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice, mice exhibit impaired motor abilities in tube and righting tests compared with control mice

impaired righting response ( J:193844 )

• as in Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

growth/size/body

decreased body weight ( J:193844 )

• as in Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice

muscle

increased skeletal muscle fiber size ( J:193844 )

• compared with Smn1^tm1Hung/Smn1^tm1Hung Tg(SMN2)2Hung mice