Mouse Genome Informatics
tg
    Tg(Prnp-HSPB1*R136W)1Kolb/0
involves: FVB/NJ
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• 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
• 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

behavior/neurological
N
• at 1 year, mice exhibit normal locomotor activity and strength (J:188399)

growth/size
N
• at 1 year, mice exhibit normal body weight (J:188399)

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