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		OMIM ID	Ref(s)
Amyotrophic Lateral Sclerosis 10, with or without Frontotemporal Dementia;		612069	J:165985