One (1) Breeding Pair of Hexb-/- Mice Mouse Model of Sandoff Diseases.
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NIH investigators have established a mouse model of Sandhoff disease through the targeted disruption of the Hexb gene. The Hexb-/- mice are deficient in both major Beta-hexosaminidase isozymes A and B. The Hexb-/- mice with their progressive and profound neurologic disturbances and their pathological findings consistent with human Sandhoff disease will be very valuable for studies of pathogenesis and for devising eventual therapy for Sandhoff disease. They are also valuable for studies on efficacy of functional enzyme replacement therapy and by gene therapy in lysosomal storage diseases with CNS involvement.