UGT8

2-hydroxyacylsphingosine 1-beta-galactosyltransferase is an enzyme that in humans is encoded by the UGT8 gene.

UGT8
Identifiers
Aliases	UGT8, CGT, UGT4, UDP glycosyltransferase 8, GalCer Synthase, Ceramide Galactosyltransferase
External IDs	OMIM: 601291; MGI: 109522; HomoloGene: 20715; GeneCards: UGT8; OMA:UGT8 - orthologs
Gene location (Human) Chr. Chromosome 4 (human) Band 4q26 Start 114,598,770 bp End 114,678,225 bp
Gene location (Mouse) Chr. Chromosome 3 (mouse) Band 3|3 G1 Start 125,658,920 bp End 125,732,268 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in inferior ganglion of vagus nerve corpus callosum subthalamic nucleus superior vestibular nucleus C1 segment Pons external globus pallidus pars reticulata ventral tegmental area pars compacta Top expressed in deep cerebellar nuclei pontine nuclei ventral tegmental area lateral geniculate nucleus globus pallidus dorsal tegmental nucleus substantia nigra lateral hypothalamus medial geniculate nucleus medial vestibular nucleus More reference expression data BioGPS n/a
Gene ontology Molecular function hexosyltransferase activity glycosyltransferase activity glucuronosyltransferase activity transferase activity N-acylsphingosine galactosyltransferase activity UDP-galactose:glucosylceramide beta-1,4-galactosyltransferase activity UDP-glycosyltransferase activity Cellular component integral component of membrane membrane intracellular membrane-bounded organelle plasma membrane Biological process lipid metabolism neuron projection morphogenesis galactosylceramide biosynthetic process glycosphingolipid metabolic process peripheral nervous system development central nervous system development paranodal junction assembly metabolism protein localization to paranode region of axon cytoskeleton organization sphingolipid metabolic process Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 7368 22239 Ensembl ENSG00000174607 ENSMUSG00000032854 UniProt Q16880 Q64676 RefSeq (mRNA) NM_001128174 NM_003360 NM_001322112 NM_001322113 NM_001322114 NM_011674 RefSeq (protein) NP_001121646 NP_001309041 NP_001309042 NP_001309043 NP_003351 NP_035804 Location (UCSC) Chr 4: 114.6 – 114.68 Mb Chr 3: 125.66 – 125.73 Mb PubMed search
Wikidata
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Function

Galactocerebrosides are abundant sphingolipids of the myelin membrane of the central nervous system and peripheral nervous system and are also present in small amounts in kidney. The key enzymatic step in the biosynthesis of galactocerebrosides consists of the transfer of galactose to ceramide catalyzed by UDP-galactose ceramide galactosyltransferase (CGT, EC 2.4.1.45). The enzyme encoded by the CGT gene is the first involved in complex lipid biosynthesis in the myelinating oligodendrocyte.

Further reading

• Yahi N, Spitalnik SL, Stefano KA, De Micco P, Gonzalez-Scarano F, Fantini J (1994). "Interferon-gamma decreases cell surface expression of galactosyl ceramide, the receptor for HIV-1 GP120 on human colonic epithelial cells". Virology. 204 (2): 550–7. doi:10.1006/viro.1994.1568. PMID 7941321.
• Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
• Bhat S, Mettus RV, Reddy EP, Ugen KE, Srikanthan V, Williams WV, Weiner DB (1993). "The galactosyl ceramide/sulfatide receptor binding region of HIV-1 gp120 maps to amino acids 206-275". AIDS Res. Hum. Retroviruses. 9 (2): 175–81. doi:10.1089/aid.1993.9.175. PMID 8457384.
• Kapitonov D, Yu RK (1997). "Cloning, characterization, and expression of human ceramide galactosyltransferase cDNA". Biochem. Biophys. Res. Commun. 232 (2): 449–53. doi:10.1006/bbrc.1997.6240. PMID 9125199.
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
• Sprong H, Kruithof B, Leijendekker R, Slot JW, van Meer G, van der Sluijs P (1998). "UDP-galactose:ceramide galactosyltransferase is a class I integral membrane protein of the endoplasmic reticulum". J. Biol. Chem. 273 (40): 25880–8. doi:10.1074/jbc.273.40.25880. PMID 9748263.
• Iida A, Saito S, Sekine A, Mishima C, Kitamura Y, Kondo K, Harigae S, Osawa S, Nakamura Y (2002). "Catalog of 86 single-nucleotide polymorphisms (SNPs) in three uridine diphosphate glycosyltransferase genes: UGT2A1, UGT2B15, and UGT8". J. Hum. Genet. 47 (10): 505–10. doi:10.1007/s100380200075. PMID 12376738.
• Sprong H, Degroote S, Nilsson T, Kawakita M, Ishida N, van der Sluijs P, van Meer G (2003). "Association of the Golgi UDP-galactose transporter with UDP-galactose:ceramide galactosyltransferase allows UDP-galactose import in the endoplasmic reticulum". Mol. Biol. Cell. 14 (8): 3482–93. doi:10.1091/mbc.E03-03-0130. PMC 181583. PMID 12925779.
• Tencomnao T, Kapitonov D, Bieberich E, Yu RK (2004). "Transcriptional regulation of the human UDP-galactose:ceramide galactosyltransferase (hCGT) gene expression: functional role of GC-box and CRE". Glycoconj. J. 20 (5): 339–51. doi:10.1023/B:GLYC.0000033630.58533.16. PMID 15229398. S2CID 38983312.