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2007 | 48 | 3 |

Tytuł artykułu

Neural stem cell implantation extends life in Niemann-Pick C1 mice

Warianty tytułu

Języki publikacji

EN

Abstrakty

EN
In order to evaluate the phenotypic effects of implanted neural stem cells (NSCs) in the mouse model of Niemann-Pick C (NPC) disease, we injected a well-characterized clone of murine NSCs into the cerebella of neonatal Npc1-/- and control mice. The implanted cells survived and were abundant in some regions of the cerebellum. Life span was lengthened in NPC mice with the implanted NSCs. However, the rate of weight gain and subsequent weight loss, resulting from neurodegeneration, was not significantly different from un-injected controls. Ataxia was measured by Rota-Rod performance. The overall rate of decline in time on the Rota-Rod was not significantly slowed down. Thus, in this small group of NPC mice, a single administration in the neonatal period of the NSCs (which were not engineered to over-express the missing gene and not directed into the parenchyma) was only partially therapeutic.

Słowa kluczowe

Wydawca

-

Rocznik

Tom

48

Numer

3

Opis fizyczny

p.269-272,fig.,ref.

Twórcy

autor
  • University of Arizona, 1501 N. Campbell Avenue, P.O.Box 245073, Tucson, AZ 85724-5073, USA
autor
autor
autor

Bibliografia

  • Baudry M, Yao Y, Simmons D, Liu J, Bi X, 2003. Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia. Exp Neurol 184: 887-903.
  • Ko DC, Milenkovic L, Beier SM, Manuel H, Buchanan J, Scott MP, 2005. Cell-autonomous death of cerebellar Purkinje neurons with autophagy in Niemann-Pick type C disease. PLOS Genet le7: 0082-0095.
  • Lacorazza HD, Flax JD, Snyder EY, Jendoubi M, 1996. Expression of human beta-hexosaminidase alpha-subunit gene (the gene defect of Tay-Sachs disease) in mouse brains upon engraftment of transduced progenitor cells. Nat Med 2: 424-429.
  • Li J, Imitola J, Snyder EY, Sidman RL, 2006. Neural stem cells rescue nervous Purkinje neurons by restoring molecular homeostasis of tissue plasminogen activator and downstream targets. J Neurosci 26: 7839-7848.
  • Ong WY, Hu CY, Soh YP, Ghoshal S, Kumar U, Pentchev PG, Patel SC, 2000. Neuronal localization of sterol regulatory element binding protein-1 in the rodent and primate brain: A light and electron microscopic immunocytochemical study. Neuroscience 97: 143-153.
  • Ong WY, Kumar U, Switzer RC, Sidhu A, Suresh G, Hu CY, Patel SC, 2001. Neurodegeneration in Niemann-Pick type C disease mice. Exp Brain Res 141: 218-231.
  • Ourednik J, Ourednik V, Lynch WP, Schachner M, Snyder EY, 2002. Neural stem cells display an inherent mechanism for rescuing dysfunctional neurons. Nat Biotechnol 20: 1103-1110.
  • Park KI, Teng YD, Snyder EY, 2002. The injured brain interacts reciprocally with neural stem cells supported by scaffolds to reconstitute lost tissue. Nature Biotechnol 20: 1111-1117.
  • Patel SC, Suresh S, Kumar U, Hu CY, Cooney A, Blanchette-Mackie EJ, et al. 1999. Localization of Niemann-Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann-Pick type C disease. Proc Natl Acad Sci USA 96: 1657-1662.
  • Reid PC, Sakashita N, Sugii S, Ohno-Iwashita Y, Shimada Y, Hickey WF, Chang TY, 2004. A novel cholesterol stain reveals early neuronal cholesterol accumulation in Niemann-Pick type C1 mouse brain. J Lipid Res 45: 582-591.
  • Rosario CM, Yandava BD, Kosaras B, Zurakowski D, Sidman RL, Snyder EY, 1997. Differentiation of engrafted multipotent neural progenitors towards replacement of missing granule neurons in meander tail cerebellum may help determine the locus of mutant gene action. Development 124: 4213-4224.
  • Sanchez-Ramos J, Song S, Dailey M, Cardozo-Pelaez F, Hazzi C, Stedeford T, et al. 2000. The X-gal caution in neural transplantation studies. Cell Transplant 9: 657-667.
  • Snyder EY, Dieter DL, Walsh C, Arnold-Aldea S, Hartwieg EA, Cepko CL, 1992. Multipotent neural cell lines can engraft and participate in development of mouse cerebellum. Cell 68: 33-51.
  • Snyder EY, Taylor RM, Wolfe JH, 1995. Neural progenitor cell engraftment corrects lysosomal storage throughout the MRS VII mouse brain. Nature 374: 367-370.
  • Snyder EY, Yoon C, Flax JD, Macklis JD, 1997. Multipotent neural precursors can differentiate toward replacement of neurons undergoing targeted apoptotic degeneration in adult mouse neocortex. PNAS 94: 11663-11668.
  • Taylor RM, Snyder EY, 1997. Widespread engraftment of neural progenitor and stem-like cells throughout the mouse brain. Transplant Proc 29: 845-847.
  • Taylor RM, Lee JP, Palacino JL, Bower KA, Li J, Vanier MT, et al. 2006. Intrinsic resistance of neural stem cells to toxic metabolites may make them well suited for cell non-autonomous disorders: evidence from a mouse model of Krabbe leukodystrophy. J Neurochem 97: 1585-1599.
  • Yandava BD, Billinghurst LL, Snyder EY, 1999. "Global" cell replacement is feasible via neural stem cell transplantation: evidence from the dysmyelinated shiverer mouse brain. Proc Nat Acad Sci USA 96: 7029-7034.
  • Yang S-R, Kim S-J, Byun K-H, Hutchinson B, Lee B-H, Michikawa M, et al. 2006. NPC1 gene deficiency leads to lack of neural stem cell self-renewal and abnormal differentiation through activation of p38 MAP kinase signaling. Stem Cells 24: 292-298.

Typ dokumentu

Bibliografia

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