Mouse Genome Informatics
cx
    Ccl3tm1Unc/Ccl3tm1Unc
Hexbtm1Rlp/Hexbtm1Rlp

involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Hexbtm1Rlp/Hexbtm1Rlp mice exhibit Sandhoff disease phenotypes that are improved in Hexbtm1Rlp/Hexbtm1Rlp Ccl3tm1Unc/Ccl3tm1Unc mice

mortality/aging
• life span of approximately 156 days (maximum 175 days)

behavior/neurological
• improved performance on a rotarod compared to mice homozygous for the Hexb allele alone
• improved relative to mice homozygous for the Hexb allele alone

growth/size/body
N
• maintained body weight through 19 weeks of age (J:90687)

immune system
• 87% reduction of TNF alpha in the spinal cord relative to levels seen in mice homozygous only for Hexb
• fewer cells infiltrating the CNS of 4 month old mice

cellular
N
• near absence of apoptosis in the central nervous system (J:90687)

nervous system
• fewer cells infiltrating the CNS of 4 month old mice

homeostasis/metabolism
• 87% reduction of TNF alpha in the spinal cord relative to levels seen in mice homozygous only for Hexb

Mouse Models of Human Disease
OMIM IDRef(s)
Sandhoff Disease 268800 J:90687