Grafted cerebellar cells in a mouse model of hereditary ataxia express IGF-I system genes and partially restore behavioral function.
Fetal grafts of normal cerebellar tissue were implanted into the cerebellum of Purkinje cell degeneration mutant mice (pcd/pcd), a model of adult-onset recessively inherited cerebello-olivary atrophy, in an attempt at correcting their cellular and motor impairment. Donor cerebellar cells engrafted in the appropriate sites, as evidenced by the pattern of expression of insulin-like growth factor-I (IGF-I) system genes. Bilateral cerebellar grafts led to an improvement of motor behaviors in balance rod tests and in the open field, providing evidence for functional integration into the atrophic mouse cerebellum and underscoring the potential of neural transplantation for counteracting the human cerebellar ataxias.
Zhang
W
W
Department of Anatomy, Indiana University School of Medicine, Indianapolis 46202, USA.
Lee
W H
WH
Triarhou
L C
LC
D000818:Animals; D016380:Brain Tissue Transplantation / Q000379:methods / Q000502:physiology*; D002524:Cerebellar Ataxia / Q000628:therapy; D002531:Cerebellum / Q000378:metabolism / Q000637:transplantation*; D016332:Fetal Tissue Transplantation / Q000502:physiology*; D007839:Functional Laterality; D015870:Gene Expression; D006085:Graft Survival; D006801:Humans; D017403:In Situ Hybridization; D007334:Insulin-Like Growth Factor I / Q000032:analysis / Q000096:biosynthesis*; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D009043:Motor Activity; D009410:Nerve Degeneration; D011689:Purkinje Cells; D012333:RNA, Messenger / Q000032:analysis / Q000096:biosynthesis; D013132:Spinocerebellar Degenerations / Q000628:therapy*
D012333:RNA, Messenger; D007334:Insulin-Like Growth Factor I