Phenotypes associated with this allele

• Allele Symbol: Tg(SOD1*G37R)42Dpr
• Allele Name: transgene insertion 42, Donald L Price
• Allele ID: MGI:3574014
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(SOD1*G37R)42Dpr/0	involves: C3H/HeJ * C57BL/6J	MGI:3814058

Genotype
MGI:3814058

tg1

• Allelic Composition: Tg(SOD1*G37R)42Dpr/0
• Genetic Background: involves: C3H/HeJ * C57BL/6J

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

nervous system

brain vacuoles ( J:69178 )

• in the choroid plexus

abnormal spinal cord morphology ( J:69178 )

• mice exhibit vacuolization in the neutropil of substantia gelatinosa and scattered in some of the fiber tracts of the ventral and lateral white matter of the spinal cord

abnormal motor neuron mitochondrial morphology ( J:69178 )

• motor neurons exhibit mitochondrial degradation

decreased motor neuron number ( J:69178 )

• at late stages

motor neuron degeneration ( J:69178 )

• motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem

• degeneration is associated with vacuole formation in both dendrites and axons of motor neurons

• mice exhibit axonal degradation in dorsal roots of motor neurons

behavior/neurological

tremors ( J:69178 )

• at 3.5 to 4 months of age, mice exhibit axial tremors

weakness ( J:69178 )

• at 3.5 to 4 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs

decreased locomotor activity ( J:69178 )

• at 3.5 to 4 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice

hindlimb paralysis ( J:69178 )

• eventually mice develop hindlimb paralysis

muscle

muscular atrophy ( J:69178 )

• at 3.5 to 4 months of age, mice exhibit muscle wasting particularly along the flanks

abnormal muscle electrophysiology ( J:69178 )

• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy

cellular

abnormal motor neuron mitochondrial morphology ( J:69178 )

• motor neurons exhibit mitochondrial degradation

growth/size/body

weight loss ( J:69178 )

• progressive

renal/urinary system

abnormal proximal convoluted tubule morphology ( J:69178 )

• mice exhibit vacuolization of epithelial cells in the proximal tubules of the kidney

integument

rough coat ( J:69178 )

• at 3.5 to 4 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:69178