The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Galc  -  galactosylceramidase

Mus musculus

Synonyms: 2310068B06Rik, A930008M05Rik, AW212969, AW413532, GALCERase, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Galc


High impact information on Galc


Chemical compound and disease context of Galc


Biological context of Galc


Anatomical context of Galc


Associations of Galc with chemical compounds


Physical interactions of Galc


Regulatory relationships of Galc


Other interactions of Galc

  • These observations are contrary to the expected outcome of Mendelian autosomal recessive single gene disorders and may also be interpreted as that the acid beta-galactosidase gene functions as a modifier gene for the phenotypic expression of genetic galactosylceramidase deficiency [13].
  • LPS was found to greatly shorten the life of twitcher and twitcher/IL-6-deficient mice compared to genotyped-matched saline-injected mice [2].
  • The possibility of the treatment of globoid cell leucodystrophy in the twitcher mouse by enzyme replacement was investigated using nerve grafts from affected animals into trembler hosts [23].
  • In twitcher chimeric mice examined after 100 postnatal days, the majority of GFP/Mac-1 double-positive cells displayed the morphological features of ramified microglia with fine delicate processes and was distributed diffusely in both gray and white matter [24].
  • The expression of the major histocompatibility complex class II antigens (Ia) was investigated in the central and peripheral nervous systems of the twitcher mouse, an authentic murine model of globoid cell leukodystrophy (Krabbe disease) in humans [25].

Analytical, diagnostic and therapeutic context of Galc


  1. The therapeutic potential of neural stem/progenitor cells in murine globoid cell leukodystrophy is conditioned by macrophage/microglia activation. Pellegatta, S., Tunici, P., Poliani, P.L., Dolcetta, D., Cajola, L., Colombelli, C., Ciusani, E., Di Donato, S., Finocchiaro, G. Neurobiol. Dis. (2006) [Pubmed]
  2. IL-6 deficiency causes enhanced pathology in Twitcher (globoid cell leukodystrophy) mice. Pedchenko, T.V., LeVine, S.M. Exp. Neurol. (1999) [Pubmed]
  3. Galactocerebrosidase-deficient oligodendrocytes maintain stable central myelin by exogenous replacement of the missing enzyme in mice. Kondo, Y., Wenger, D.A., Gallo, V., Duncan, I.D. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  4. Quantification of galactosylsphingosine in the twitcher mouse using electrospray ionization-tandem mass spectrometry. Whitfield, P.D., Sharp, P.C., Taylor, R., Meikle, P. J. Lipid Res. (2001) [Pubmed]
  5. Hematopoietic cell transplantation in the twitcher mouse. The effects of pretransplant conditioning with graded doses of busulfan. Yeager, A.M., Shinn, C., Shinohara, M., Pardoll, D.M. Transplantation (1993) [Pubmed]
  6. Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy. Matsushima, G.K., Taniike, M., Glimcher, L.H., Grusby, M.J., Frelinger, J.A., Suzuki, K., Ting, J.P. Cell (1994) [Pubmed]
  7. Enzyme replacement in grafted nerve of twitcher mouse. Scaravilli, F., Suzuki, K. Nature (1983) [Pubmed]
  8. Dysfunction of peroxisomes in twitcher mice brain: a possible mechanism of psychosine-induced disease. Haq, E., Contreras, M.A., Giri, S., Singh, I., Singh, A.K. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  9. Progressive dysfunction of twitcher Schwann cells is evaluated better in vitro than in vivo. Komiyama, A., Suzuki, K. Brain Res. (1994) [Pubmed]
  10. Prostaglandin D2-mediated microglia/astrocyte interaction enhances astrogliosis and demyelination in twitcher. Mohri, I., Taniike, M., Taniguchi, H., Kanekiyo, T., Aritake, K., Inui, T., Fukumoto, N., Eguchi, N., Kushi, A., Sasai, H., Kanaoka, Y., Ozono, K., Narumiya, S., Suzuki, K., Urade, Y. J. Neurosci. (2006) [Pubmed]
  11. Krabbe disease: psychosine-mediated activation of phospholipase A2 in oligodendrocyte cell death. Giri, S., Khan, M., Rattan, R., Singh, I., Singh, A.K. J. Lipid Res. (2006) [Pubmed]
  12. L-cycloserine slows the clinical and pathological course in mice with globoid cell leukodystrophy (twitcher mice). LeVine, S.M., Pedchenko, T.V., Bronshteyn, I.G., Pinson, D.M. J. Neurosci. Res. (2000) [Pubmed]
  13. Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency). Tohyama, J., Vanier, M.T., Suzuki, K., Ezoe, T., Matsuda, J., Suzuki, K. Hum. Mol. Genet. (2000) [Pubmed]
  14. Perineuronal oligodendrocytes protect against neuronal apoptosis through the production of lipocalin-type prostaglandin D synthase in a genetic demyelinating model. Taniike, M., Mohri, I., Eguchi, N., Beuckmann, C.T., Suzuki, K., Urade, Y. J. Neurosci. (2002) [Pubmed]
  15. Decreased fatty acylation of myelin proteolipid protein in the twitcher mouse. Yoshimura, T., Kobayashi, T., Mitsuo, K., Goto, I. J. Neurochem. (1989) [Pubmed]
  16. Accumulation of galactosylsphingosine (psychosine) does not interfere with phosphorylation and methylation of myelin basic protein in the twitcher mouse. Yoshimura, T., Kobayashi, T., Shinnoh, N., Goto, I. Neurochem. Res. (1990) [Pubmed]
  17. Murine leukodystrophies as tools to study myelinogenesis in normal and pathological conditions. Matthieu, J.M., Omlin, F.X. Neuropediatrics. (1984) [Pubmed]
  18. Glycolipids of the spinal cord, sciatic nerve, and systemic organs of the twitcher mouse. Igisu, H., Suzuki, K. J. Neuropathol. Exp. Neurol. (1984) [Pubmed]
  19. Suppressed UDP-galactose: ceramide galactosyltransferase and myelin protein mRNA in twitcher mouse brain. Taniike, M., Marcus, J.R., Nishigaki, T., Fujita, N., Popko, B., Suzuki, K., Suzuki, K. J. Neurosci. Res. (1998) [Pubmed]
  20. Expression of major histocompatibility complex class I antigens in the demyelinating twitcher CNS and PNS. Taniike, M., Marcus, J.R., Popko, B., Suzuki, K. J. Neurosci. Res. (1997) [Pubmed]
  21. Lipocalin-type prostaglandin D synthase is up-regulated in oligodendrocytes in lysosomal storage diseases and binds gangliosides. Mohri, I., Taniike, M., Okazaki, I., Kagitani-Shimono, K., Aritake, K., Kanekiyo, T., Yagi, T., Takikita, S., Kim, H.S., Urade, Y., Suzuki, K. J. Neurochem. (2006) [Pubmed]
  22. The twitcher mouse: myelinogenesis in organotypic culture. Bourque, E.A., Bornstein, M.B., Peterson, E.R., Suzuki, K. Brain Res. (1983) [Pubmed]
  23. Improved myelination in nerve grafts from the leucodystrophic twitcher into trembler mice: evidence for enzyme replacement. Scaravilli, F., Jacobs, J.M. Brain Res. (1982) [Pubmed]
  24. Distribution and characterization of GFP(+) donor hematogenous cells in Twitcher mice after bone marrow transplantation. Wu, Y.P., McMahon, E., Kraine, M.R., Tisch, R., Meyers, A., Frelinger, J., Matsushima, G.K., Suzuki, K. Am. J. Pathol. (2000) [Pubmed]
  25. MHC class II antigen expression and T-cell infiltration in the demyelinating CNS and PNS of the twitcher mouse. Ohno, M., Komiyama, A., Martin, P.M., Suzuki, K. Brain Res. (1993) [Pubmed]
  26. AAV2/5 vector expressing galactocerebrosidase ameliorates CNS disease in the murine model of globoid-cell leukodystrophy more efficiently than AAV2. Lin, D., Fantz, C.R., Levy, B., Rafi, M.A., Vogler, C., Wenger, D.A., Sands, M.S. Mol. Ther. (2005) [Pubmed]
  27. Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse. Yeager, A.M., Brennan, S., Tiffany, C., Moser, H.W., Santos, G.W. Science (1984) [Pubmed]
  28. The twitcher mouse: a model for Krabbe disease and for experimental therapies. Suzuki, K., Suzuki, K. Brain Pathol. (1995) [Pubmed]
WikiGenes - Universities