Mouse Genome Informatics
tg
    Tg(Prnp-HSPB1*R136W)1Kolb/0
involves: FVB/NJ
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• at 1 year, mice exhibit axonal pathology and repair with increased number of Schmidt-Lanterman incisures, myelin corrugation, infoldings and outfoldings and fibers with increased myelin thickness, density of axoplasm, axonal swelling and degeneration, and neurofilament density
• at 1 year, mice exhibit decreased sciatic motor amplitude compared with control mice
• however, mice exhibit normal tail motor and orthodromic tail sensory amplitudes and conduction velocity

behavior/neurological
N
• at 1 year, mice exhibit normal locomotor activity and strength

growth/size/body
N
• at 1 year, mice exhibit normal body weight

Mouse Models of Human Disease
OMIM IDRef(s)
Neuronopathy, Distal Hereditary Motor, Type IIB; HMN2B 608634 J:188399