Phenotypes Associated with This Genotype

Genotype
MGI:4835659

cx19

• Allelic Composition: Bax^tm1Sjk/Bax^tm1Sjk; Tg(SOD1*G93A)1Gur/0
• Genetic Background: involves: 129X1/SvJ * C57BL/6 * SJL

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

mortality/aging

premature death ( J:111890 )

• live 14.6% longer than Tg(SOD1*G93A)1Gur mice

behavior/neurological

impaired coordination ( J:111890 )

• delayed onset of motor decline

• maintain 11 rpm on the rotarod for a longer period of their lifespan than Tg(SOD1*G93A)1Gur mice

nervous system

gliosis ( J:111890 )

• delayed reactive gliosis (P100 vs P40, compared with Tg(SOD1*G93A)1Gur mice)

abnormal motor neuron morphology ( J:111890 )

• atrophic axons of the lumbar motoneurons (MNs)

• the number of Slc18a3 mRNA-expressing MNs is not reduced in the entire lumbar spinal cord at the end-stage, compared with age-matched Bax^tm1Sjk homozygous controls

• many small groups of unmyelinated axons wrapped in glial cell processes in the end-stage fourth lumbar ventral root (L4 VR)

• higher levels of partially innervated synapses in end-stage delayed accumulation of mutant SOD1 in MNs (P40 vs P30, compared with Tg(SOD1*G93A)1Gur mice)

• delayed accumulation of mutant SOD1 in MNs (P40 vs P30, compared with Tg(SOD1*G93A)1Gur mice)

abnormal motor neuron innervation pattern ( J:111890 )

• decreased rate of neuromuscular denervation

• more medial gastrocnemii (MGs) axons and innervated

• neuromuscular junctions (NMJs) than Tg(SOD1*G93A)1Gur mutants at postnatal day 100 (P100)

• reduction of innervated synapses in the MG at P45, delayed onset compared with Tg(SOD1*G93A)1Gur mice

• contain more fully innervated NMJs at subsequent ages than Tg(SOD1*G93A)1Gur mutants, until P120

decreased motor neuron number ( J:111890 )

• delayed loss of both distal and proximal myelinated axons

• reduction of myelinated axons in end-stage MG nerve and L4 VR

cellular

abnormal mitochondrial morphology ( J:111890 )

• presence of vacuolated mitochondria in the ventral spinal cord at P25

• massive vacuolization in the lumbar ventral spinal cord at P100