Gene Symbol: Ugt8a
Description: UDP galactosyltransferase 8A
Alias: AI850488, AW455908, Cgt, Ugt8, mCerGT, 2-hydroxyacylsphingosine 1-beta-galactosyltransferase, UDP-galactose-ceramide galactosyltransferase 8A, UDP-glucuronosyltransferase 8, ceramide UDP-galactosyltransferase, cerebroside synthase
Species: mouse
Products:     Ugt8a

Top Publications

  1. Honke K, Hirahara Y, Dupree J, Suzuki K, Popko B, Fukushima K, et al. Paranodal junction formation and spermatogenesis require sulfoglycolipids. Proc Natl Acad Sci U S A. 2002;99:4227-32 pubmed
    ..These data show a critical role for sulfoglycolipids in myelin function and spermatogenesis. ..
  2. Zöller I, Bussow H, Gieselmann V, Eckhardt M. Oligodendrocyte-specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT-deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide level. Glia. 2005;52:190-8 pubmed
    ..Mice lacking GalC due to ceramide galactosyltransferase (CGT) deficiency form unstable and functionally affected myelin and exhibit a progressive demyelination, accompanied by ..
  3. Saadat L, Dupree J, Kilkus J, Han X, Traka M, Proia R, et al. Absence of oligodendroglial glucosylceramide synthesis does not result in CNS myelin abnormalities or alter the dysmyelinating phenotype of CGT-deficient mice. Glia. 2010;58:391-8 pubmed publisher
    ..HFA-GlcCer) accumulation in UDP-galactose:ceramide galactosyltransferase (encoded by Cgt, also known as Ugt8a) deficient mice...
  4. Poliak S, Gollan L, Salomon D, Berglund E, Ohara R, Ranscht B, et al. Localization of Caspr2 in myelinated nerves depends on axon-glia interactions and the generation of barriers along the axon. J Neurosci. 2001;21:7568-75 pubmed
    ..during development of peripheral nerves of normal and galactolipids-deficient [ceramide galactosyl transferase (CGT)-/-] mice...
  5. Marcus J, Dupree J, Popko B. Effects of galactolipid elimination on oligodendrocyte development and myelination. Glia. 2000;30:319-28 pubmed
    ..galactocerebroside and sulfatide, which require the enzyme UDP-galactose:ceramide galactosyltransferase (CGT) for their synthesis, are among the most prevalent molecules in the myelin sheath...
  6. Dupree J, Popko B. Genetic dissection of myelin galactolipid function. J Neurocytol. 1999;28:271-9 pubmed
    ..Recently, the isolation of the gene that encodes UDP-galactose:ceramide galactosyltransferase (CGT), the enzyme that catalyzes an essential step in the synthetic pathway of GalC and sulfatide, has enabled the ..
  7. Bosio A, Bussow H, Adam J, Stoffel W. Galactosphingolipids and axono-glial interaction in myelin of the central nervous system. Cell Tissue Res. 1998;292:199-210 pubmed
    The myelin of central and peripheral nervous system of UDP-galactose-ceramide galactosyltransferase deficient mice (cgt-/-) is completely depleted of its major lipid constituents, galactocerebrosides and sulfatides...
  8. Dupree J, Coetzee T, Blight A, Suzuki K, Popko B. Myelin galactolipids are essential for proper node of Ranvier formation in the CNS. J Neurosci. 1998;18:1642-9 pubmed
    ..with a disruption in the gene that encodes the biosynthetic enzyme UDP-galactose:ceramide galactosyl transferase (CGT) are incapable of synthesizing these lipids yet form myelin sheaths that exhibit major and minor dense lines with ..
  9. Bosio A, Binczek E, Stoffel W. Functional breakdown of the lipid bilayer of the myelin membrane in central and peripheral nervous system by disrupted galactocerebroside synthesis. Proc Natl Acad Sci U S A. 1996;93:13280-5 pubmed
    ..We have generated a UDP-galactose ceramide galactosyltransferase (CGT) null mutant mouse (cgt-/-) with CNS and PNS myelin completely depleted of GalC and derived sGalC...

More Information


  1. Bosio A, Binczek E, Haupt W, Stoffel W. Composition and biophysical properties of myelin lipid define the neurological defects in galactocerebroside- and sulfatide-deficient mice. J Neurochem. 1998;70:308-15 pubmed
    ..cerebrosides and sulfatides by generating a ceramide galactosyltransferase null allelic mouse line (cgt-/-)...
  2. Coetzee T, Suzuki K, Nave K, Popko B. Myelination in the absence of galactolipids and proteolipid proteins. Mol Cell Neurosci. 1999;14:41-51 pubmed
    ..These data indicate that the galactolipids and PLP/DM20 are not required for intraperiod line formation, but they suggest a role for these molecules in mediating myelin compaction and in maintaining the integrity of the cerebellum. ..
  3. Dupree J, Girault J, Popko B. Axo-glial interactions regulate the localization of axonal paranodal proteins. J Cell Biol. 1999;147:1145-52 pubmed
  4. Popko B. Myelin galactolipids: mediators of axon-glial interactions?. Glia. 2000;29:149-53 pubmed
    ..These data indicate that the galactolipids play an essential role in axon-glial interactions and node of Ranvier formation. ..
  5. Coetzee T, Fujita N, DuPree J, Shi R, Blight A, Suzuki K, et al. Myelination in the absence of galactocerebroside and sulfatide: normal structure with abnormal function and regional instability. Cell. 1996;86:209-19 pubmed
    ..We have generated mice lacking the enzyme UDP-galactose:ceramide galactosyltransferase (CGT), which is required for GalC synthesis...
  6. Hennet T, Dinter A, Kuhnert P, Mattu T, Rudd P, Berger E. Genomic cloning and expression of three murine UDP-galactose: beta-N-acetylglucosamine beta1,3-galactosyltransferase genes. J Biol Chem. 1998;273:58-65 pubmed
    ..The identification of beta3GalT genes emphasizes the structural diversity present in the galactosyltransferase gene family. ..
  7. Jahng A, Maricic I, Aguilera C, Cardell S, Halder R, Kumar V. Prevention of autoimmunity by targeting a distinct, noninvariant CD1d-reactive T cell population reactive to sulfatide. J Exp Med. 2004;199:947-57 pubmed
  8. Zhuang L, Wang B, Shinder G, Shivji G, Mak T, Sauder D. TNF receptor p55 plays a pivotal role in murine keratinocyte apoptosis induced by ultraviolet B irradiation. J Immunol. 1999;162:1440-7 pubmed
    ..Our observations support the notion that TNF-alpha is involved in UVB-induced keratinocyte apoptosis, and demonstrate that p55 receptor signaling plays a pivotal role in this event. ..
  9. Meixner M, Jungnickel J, Grothe C, Gieselmann V, Eckhardt M. Myelination in the absence of UDP-galactose:ceramide galactosyl-transferase and fatty acid 2 -hydroxylase. BMC Neurosci. 2011;12:22 pubmed publisher
    ..importance of GalCer and sulfatide has been validated using UDP-galactose:ceramide galactosyltransferase-deficient (Cgt-/-) mice, which are impaired in myelin maintenance. These mice, however, are still able to form compact myelin...
  10. Haupt W, Stoffel W. Nerve conduction velocity measurements reveal the functional deficit in ceramide galactosyl transferase-deficient (cgt-/-) mice. J Neurol Sci. 2004;217:83-8 pubmed
    Biochemical and ultrastructural studies of ceramide galactosyltransferase (CGT) in a CGT-deficient mouse line (cgt-/-) were complemented by nerve conduction velocity (NCV) measurements in motor nerves (sciatic nerve in the hind limbs) of ..
  11. Stanic A, De Silva A, Park J, Sriram V, Ichikawa S, Hirabyashi Y, et al. Defective presentation of the CD1d1-restricted natural Va14Ja18 NKT lymphocyte antigen caused by beta-D-glucosylceramide synthase deficiency. Proc Natl Acad Sci U S A. 2003;100:1849-54 pubmed
    ..These findings suggest that beta-D-GlcCer may play an important role in generating and/or loading a natural Va14Ja18 NKT antigen. ..
  12. Katayama Y, Battista M, Kao W, Hidalgo A, Peired A, Thomas S, et al. Signals from the sympathetic nervous system regulate hematopoietic stem cell egress from bone marrow. Cell. 2006;124:407-21 pubmed
    ..UDP-galactose ceramide galactosyltransferase-deficient (Cgt(-/-)) mice exhibit aberrant nerve conduction and display virtually no HSPC egress from BM following granulocyte ..
  13. Citron B, Ratzlaff K, Smirnova I, Festoff B. Protease nexin I (PNI) in mouse brain is expressed from the same gene as in seminal vesicle. J Mol Neurosci. 1996;7:183-91 pubmed
    ..The PNI cDNAs generated will serve as useful probes for the continued characterization of the serpin:protease balance as it relates to nerve cell function. ..
  14. Traka M, Dupree J, Popko B, Karagogeos D. The neuronal adhesion protein TAG-1 is expressed by Schwann cells and oligodendrocytes and is localized to the juxtaparanodal region of myelinated fibers. J Neurosci. 2002;22:3016-24 pubmed
    ..The CNS of the UDP-galactose ceramide galactosyl transferase(-/-) (CGT(-/-)) mouse mutants, which do not synthesize the abundant galactolipids of myelin, display severely disrupted ..
  15. Ezoe T, Vanier M, Oya Y, Popko B, Tohyama J, Matsuda J, et al. Twitcher mice with only a single active galactosylceramide synthase gene exhibit clearly detectable but therapeutically minor phenotypic improvements. J Neurosci Res. 2000;59:179-87 pubmed
    Cross-breeding of mouse mutants, each defective in either synthesis (CGT knockout) or degradation (twitcher) of galactosylceramide, generates hybrids with a genotype of galc -/-, cgt +/-, in addition to doubly deficient mice...
  16. Lullmann Rauch R, Matzner U, Franken S, Hartmann D, Gieselmann V. Lysosomal sulfoglycolipid storage in the kidneys of mice deficient for arylsulfatase A (ASA) and of double-knockout mice deficient for ASA and galactosylceramide synthase. Histochem Cell Biol. 2001;116:161-9 pubmed
    ..ASA-/-) and (b) synthesizing the major sulfolipid S-GalCer because of deficiency for galactosylceramide synthase (CGT), with the aim to search for additional ASA substrates...
  17. Fewou S, Fernandes A, Stockdale K, Francone V, Dupree J, Rosenbluth J, et al. Myelin protein composition is altered in mice lacking either sulfated or both sulfated and non-sulfated galactolipids. J Neurochem. 2010;112:599-610 pubmed publisher
    ..Mice, unable to synthesize GalCer and sulfatide (CGT(null)) or sulfatide alone (CST(null)), exhibit disorganized paranodal structures and progressive dysmyelination...
  18. Tadano Aritomi K, Hikita T, Fujimoto H, Suzuki K, Motegi K, Ishizuka I. Kidney lipids in galactosylceramide synthase-deficient mice. Absence of galactosylsulfatide and compensatory increase in more polar sulfoglycolipids. J Lipid Res. 2000;41:1237-43 pubmed
    UDP-galactose:ceramide galactosyltransferase (CGT) catalyzes the final step in the synthesis of galactosylceramide (GalCer)...
  19. Weng Q, Chen Y, Wang H, Xu X, Yang B, He Q, et al. Dual-mode modulation of Smad signaling by Smad-interacting protein Sip1 is required for myelination in the central nervous system. Neuron. 2012;73:713-28 pubmed publisher
    ..Thus, our findings reveal that Sip1-mediated antagonism of inhibitory signaling is critical for promoting CNS myelination and point to new mediators for myelin repair...
  20. Kagawa T, Oba A, Okumura S, Ikenaka K. Localization of mRNA for UDP-galactose: ceramide galactosyltransferase in the brain during mouse development. Dev Neurosci. 1996;18:309-18 pubmed
    UDP-galactose:ceramide galactosyltransferase (CGT) is considered to be a key enzyme in the biosynthesis of galactocerebroside (GalC), a frequently used marker for oligodendrocytes...
  21. Coetzee T, Li X, Fujita N, Marcus J, Suzuki K, Francke U, et al. Molecular cloning, chromosomal mapping, and characterization of the mouse UDP-galactose:ceramide galactosyltransferase gene. Genomics. 1996;35:215-22 pubmed
    UDP-galactose:ceramide galactosyltransferase (CGT) (EC catalyzes the final step in the synthesis of galactocerebroside, a glycosphingolipid characteristically abundant in myelin...
  22. Chen Y, Wu H, Wang S, Koito H, Li J, Ye F, et al. The oligodendrocyte-specific G protein-coupled receptor GPR17 is a cell-intrinsic timer of myelination. Nat Neurosci. 2009;12:1398-406 pubmed publisher
  23. Bosio A, Binczek E, Stoffel W. Molecular cloning and characterization of the mouse CGT gene encoding UDP-galactose ceramide-galactosyltransferase (cerebroside synthetase). Genomics. 1996;35:223-6 pubmed
    UDP-galactose ceramide galactosyltransferase, CGT, EC 2.4.1...
  24. Traka M, Wollmann R, Cerda S, Dugas J, Barres B, Popko B. Nur7 is a nonsense mutation in the mouse aspartoacylase gene that causes spongy degeneration of the CNS. J Neurosci. 2008;28:11537-49 pubmed publisher
    ..heterozygous for a null allele of the gene that encodes the enzyme UDP-galactose:ceramide galactosyltransferase (Cgt), which is responsible for catalyzing the synthesis of the abundant myelin galactolipids...
  25. Kongmanas K, Xu H, Yaghoubian A, Franchini L, Panza L, Ronchetti F, et al. Quantification of seminolipid by LC-ESI-MS/MS-multiple reaction monitoring: compensatory levels in Cgt(+/?) mice. J Lipid Res. 2010;51:3548-58 pubmed publisher
    ..Using ESI-MS/MS MRM, C16:0-alkyl/C16:0-acyl SGG of Cgt(+/?) mice was quantified to be 406.06 ± 23.63 ?g/g testis and 0.13 ± 0...
  26. Garcia Fresco G, Sousa A, Pillai A, Moy S, Crawley J, Tessarollo L, et al. Disruption of axo-glial junctions causes cytoskeletal disorganization and degeneration of Purkinje neuron axons. Proc Natl Acad Sci U S A. 2006;103:5137-42 pubmed
    ..Mice deficient in either the axonal protein NCP1 or the glial ceramide galactosyltransferase (CGT) display disruptions in AGJs and severe ataxia...
  27. Small C, Shima J, Uzumcu M, Skinner M, Griswold M. Profiling gene expression during the differentiation and development of the murine embryonic gonad. Biol Reprod. 2005;72:492-501 pubmed
  28. Katayama Y, Frenette P. Galactocerebrosides are required postnatally for stromal-dependent bone marrow lymphopoiesis. Immunity. 2003;18:789-800 pubmed
    ..Here, we show that mice lacking the key enzyme to generate GCs, UDP-galactose:ceramide galactosyltransferase (CGT(-/-)), exhibit severe postnatal atrophy of all lymphoid organs, owing to a maturational arrest before the pro-B/T ..
  29. Takamiya K, Okada M, Inoue M, Fukumoto S, Furukawa K. Novel functions of complex carbohydrates elucidated by the mutant mice of glycosyltransferase genes. Biochim Biophys Acta. 2001;1525:1-12 pubmed
  30. Popko B, Dupree J, Coetzee T, Suzuki K. Genetic analysis of myelin galactolipid function. Adv Exp Med Biol. 1999;468:237-44 pubmed
    The CGT enzyme is responsible for catalyzing the final step in GalC synthesis...
  31. Coetzee T, Dupree J, Popko B. Demyelination and altered expression of myelin-associated glycoprotein isoforms in the central nervous system of galactolipid-deficient mice. J Neurosci Res. 1998;54:613-22 pubmed
    ..Taken together, these findings indicate that GalC and sulfatide are required for the long-term maintenance of myelin and that their absence may have subtle effects on the development of oligodendrocytes. ..
  32. Schafer D, Bansal R, Hedstrom K, Pfeiffer S, Rasband M. Does paranode formation and maintenance require partitioning of neurofascin 155 into lipid rafts?. J Neurosci. 2004;24:3176-85 pubmed
    ..Together, these results suggest that trans interactions between oligodendroglial NF-155 and axonal ligands result in cross-linking, stabilization, and formation of paranodal lipid raft assemblies. ..
  33. Dours Zimmermann M, Maurer K, Rauch U, Stoffel W, Fassler R, Zimmermann D. Versican V2 assembles the extracellular matrix surrounding the nodes of ranvier in the CNS. J Neurosci. 2009;29:7731-42 pubmed publisher
    ..The conjoint loss of tenascin-R and phosphacan from the perinodal matrix provide strong evidence that versican V2, possibly controlled by a nodal receptor, organizes the extracellular matrix assembly in vivo. ..
  34. Marcus J, Dupree J, Popko B. Myelin-associated glycoprotein and myelin galactolipids stabilize developing axo-glial interactions. J Cell Biol. 2002;156:567-77 pubmed
    ..These data indicate that distinct molecular mechanisms are responsible for the formation and maintenance of axo-glial interactions. ..
  35. Spörkel O, Uschkureit T, Bussow H, Stoffel W. Oligodendrocytes expressing exclusively the DM20 isoform of the proteolipid protein gene: myelination and development. Glia. 2002;37:19-30 pubmed
  36. Ezoe T, Vanier M, Oya Y, Popko B, Tohyama J, Matsuda J, et al. Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide. J Neurosci Res. 2000;59:170-8 pubmed cross-breeding twitcher mice and galactosylceramide synthase (UDP-galactose:ceramide galactosyltransferase, CGT) knockout mice...
  37. Yu Y, Chen Y, Kim B, Wang H, Zhao C, He X, et al. Olig2 targets chromatin remodelers to enhancers to initiate oligodendrocyte differentiation. Cell. 2013;152:248-61 pubmed publisher