Phenotypes Associated with This Genotype

Genotype
MGI:5484562

• Allelic Composition: Smn1^tm1Hung/Smn1^tm1Hung; Tg(SMN2)2Hung/0
• Genetic Background: B6N.Cg-Smn1^tm1Hung Tg(SMN2)2Hung

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

mortality/aging

premature death ( J:193844 )

• mice exhibit reduced survival compared with Smn1^tm1Hung/Smn1⁺ Tg(SMN2)2Hung mice

• Background Sensitivity: mice on a C57BL/6N background exhibit increased survival compared with mice on an FVB/N background but not as much as on a mixed background

nervous system

abnormal motor neuron morphology ( J:193844 )

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

abnormal proprioceptive neuron morphology ( J:193844 )

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

abnormal neuromuscular synapse morphology ( J:193844 )

• mice exhibit reduced axon input and endplate side compared with Smn1^tm1Hung/Smn1⁺ Tg(SMN2)2Hung mice

behavior/neurological

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

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

impaired righting response ( J:193844 )

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

muscle

decreased skeletal muscle fiber size ( J:193844 )

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

growth/size/body

decreased body size ( J:193844 )

• mice on a C57BL/6N background are smaller than mice on an FVB/N or mixed background

decreased body weight ( J:193844 )

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