Phenotypes Associated with This Genotype

Genotype
MGI:4839655

• Allelic Composition: Tg(Prnp-TARDBP)4Jlel/0
• Genetic Background: involves: C57BL/6 * CD-1 * SJL

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

mortality/aging

premature death ( J:165985 )

• mean survival is 109 days

muscle

increased variability of skeletal muscle fiber size ( J:165985 )

hypotonia ( J:165985 )

muscle weakness ( J:165985 )

• mice exhibit weakness that manifests as external rotation in one hindlimb followed by development of severe bilateral proximal weakness and hypotonia unlike wild-type mice

myopathy ( J:165985 )

• muscles exhibit variation in muscle fiber size, prominent myopathic grouping, and disorganization of the myofiber internal architecture unlike wild-type muscles

behavior/neurological

abnormal motor coordination/balance ( J:165985 )

• mice exhibit late onset, progressive motor defects compared with wild-type mice

decreased grip strength ( J:165985 )

short stride length ( J:165985 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 10		DOID:0060201	OMIM:612069	J:165985