Phenotypes Associated with This Genotype

Genotype
MGI:4440461

• Allelic Composition: Cybb^tm1Din/Y; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: B6.Cg-Cybb^tm1Din Tg(SOD1*G93A)^dl1Gur

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

mortality/aging

premature death ( J:111782 )

♂ • life span improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur

behavior/neurological

abnormal righting response ( J:111782 )

♂ • used as a measure of paralysis

♂ • improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur

paralysis ( J:111782 )

♂ • reached end stage paralysis later than mice only carrying Tg(SOD1*G93A)^dl1Gur

nervous system

neurodegeneration ( J:111782 )

♂ • improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur

axon degeneration ( J:111782 )

♂ • 50% more anterior horn motor neurons in the anterior horn of the spinal cord than in mice only carrying Tg(SOD1*G93A)^dl1Gur

♂ • more myelinated axons in the 5th lumbar anterior roots than in mice only carrying Tg(SOD1*G93A)^dl1Gur

♂ • more innervated end plates than in mice only carrying Tg(SOD1*G93A)^dl1Gur

muscle

increased skeletal muscle fiber size ( J:111782 )

♂ • in the fibularis and peroneus longus

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:111782