Mouse Genome Informatics
hm
    Ndrg1tm1Myta/Ndrg1tm1Myta
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• some mice die between 1 to 10 months

behavior/neurological
• observed upon tail suspension
• in the hindlimb at 12 weeks of age
• hindlimb weakness first exhibited at 3 months of age and increasing with time
• mice were dragging their hindlimbs by 1 year of age and exhibited hindlimb muscle atrophy
• hindlimb dragging at 1 year of age

nervous system
• severe degeneration of the sciatic nerve observed at 3 months and progressing with age
• electron microscopy revealed onion bulb pathology with Schwann cell processes, thin myelin sheaths, endoneurial collagenization, and infiltration of macrophages
• first observed at 5 weeks and progressing with age (J:89888)
• due to defects in the maintenance of myelin structure rather than impaired Schwann cell proliferation or initial myelination (J:89888)
• less severe than in Ndrg1str homozygotes (J:172770)
• at 30 weeks of age in motor and sensory nerves

muscle
• in the hind leg at 1 year of age

growth/size/body

vision/eye
• as young as 3 weeks of age

Mouse Models of Human Disease
OMIM IDRef(s)
Charcot-Marie-Tooth Disease, Type 4D; CMT4D 601455 J:89888