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Showing Protein Beta-1,4-galactosyltransferase 6 (HMDBP07258)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 47 metabolites
Identification
HMDB Protein ID HMDBP07258
Secondary Accession Numbers
  • 12877
Name Beta-1,4-galactosyltransferase 6
Synonyms
  1. Beta-1,4-GalTase 6
  2. Beta4Gal-T6
  3. LacCer synthase
  4. Lactosylceramide synthase
  5. UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 6
  6. UDP-Gal:glucosylceramide beta-1,4-galactosyltransferase
  7. UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 6
  8. b4Gal-T6
Gene Name B4GALT6
Protein Type Unknown
Biological Properties
General Function Involved in transferase activity, transferring glycosyl groups
Specific Function Required for the biosynthesis of glycosphingolipids.
Pathways
  • Fabry disease
  • Gaucher Disease
  • Globoid Cell Leukodystrophy
  • Krabbe disease
  • Metachromatic Leukodystrophy (MLD)
  • protein glycosylation
  • sphingolipid metabolism
  • Starch and sucrose metabolism
Reactions
UDP-L-rhamnose + Glucosylceramide → Uridine 5'-diphosphate + LacCer(d18:1/24:0) details
Uridine diphosphategalactose + beta-D-glucosyl-(1<->1)-ceramide → Uridine 5'-diphosphate + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide details
beta-D-Glucosyl-(1<->1)-ceramide + Uridine diphosphategalactose → beta-D-Galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide + Uridine 5'-diphosphate details
Glucocerebroside + UDP-D-galactose → Lactosylceramide + UDP details
GO Classification
Biological Process
keratan sulfate biosynthetic process
post-translational protein modification
protein N-linked glycosylation via asparagine
Cellular Component
Golgi cisterna membrane
integral to membrane
Golgi membrane
Function
catalytic activity
transferase activity
transferase activity, transferring glycosyl groups
Molecular Function
metal ion binding
UDP-galactose:glucosylceramide beta-1,4-galactosyltransferase activity
galactosyltransferase activity
Process
metabolic process
primary metabolic process
carbohydrate metabolic process
Cellular Location
  1. Single-pass type II membrane protein
  2. Golgi apparatus
  3. Golgi stack membrane
Gene Properties
Chromosome Location 18
Locus 18q11
SNPs B4GALT6
Gene Sequence 
>1149 bp
ATGTCTGTGCTCAGGCGGATGATGCGGGTTTCCAATCGCTCTCTCCTCGCCTTCATCTTC
TTCTTCTCCCTCTCTTCGTCCTGTCTGTACTTCATCTATGTGGCCCCAGGCATCGCCAAC
ACATATCTCTTTATGGTACAAGCTCGAGGTATAATGTTGAGAGAAAATGTGAAAACAATA
GGTCATATGATCAGGCTGTACACAAATAAAAACAGTACGCTCAACGGTACAGATTATCCC
GAAGGCAATAATTCAAGTGATTATCTTGTTCAAACAACAACGTATCTCCCGGAAAACTTC
ACATACTCACCATACCTCCCCTGTCCAGAAAAGCTGCCTTATATGCGAGGATTCCTCAAT
GTCAATGTAAGCGAAGTCAGTTTTGATGAAATTCATCAACTCTTCTCCAAGGATTTAGAT
ATTGAGCCAGGGGGTCATTGGAGGCCAAAAGACTGTAAACCCAGATGGAAGGTGGCAGTT
CTCATTCCTTTCCGTAATCGCCATGAACATCTTCCAATTTTTTTCTTACATCTGATTCCA
ATGCTCCAGAAGCAGCGGCTGGAATTTGCGTTTTATGTCATTGAACAGACTGGCACACAA
CCTTTTAACCGTGCGATGCTTTTCAATGTGGGCTTCAAAGAGGCCATGAAAGACAGTGTC
TGGGACTGTGTAATCTTCCACGATGTGGATCATCTACCTGAAAATGACCGGAACTATTAC
GGATGTGGAGAAATGCCACGTCATTTTGCTGCAAAGCTGGATAAATACATGTATATTCTT
CCATATAAAGAATTTTTTGGTGGTGTAAGTGGGCTGACAGTGGAACAATTTAGAAAGATC
AATGGTTTTCCTAATGCCTTCTGGGGATGGGGAGGAGAAGATGATGACCTTTGGAACAGA
GTTCACTATGCTGGATATAATGTAACCAGACCAGAGGGAGACTTAGGAAAATACAAGTCA
ATTCCTCATCACCATAGAGGTGAAGTCCAGTTTTTAGGACGGTATAAATTACTAAGGTAT
TCCAAGGAGCGTCAGTACATCGATGGACTGAACAATTTAATATATAGGCCAAAAATACTG
GTTGATAGGTTGTATACAAACATATCTGTAAACCTCATGCCAGAGTTAGCTCCAATCGAA
GACTATTAA
Protein Properties
Number of Residues 382
Molecular Weight 44913.315
Theoretical pI 8.425
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence 
>Beta-1,4-galactosyltransferase 6
MSVLRRMMRVSNRSLLAFIFFFSLSSSCLYFIYVAPGIANTYLFMVQARGIMLRENVKTI
GHMIRLYTNKNSTLNGTDYPEGNNSSDYLVQTTTYLPENFTYSPYLPCPEKLPYMRGFLN
VNVSEVSFDEIHQLFSKDLDIEPGGHWRPKDCKPRWKVAVLIPFRNRHEHLPIFFLHLIP
MLQKQRLEFAFYVIEQTGTQPFNRAMLFNVGFKEAMKDSVWDCVIFHDVDHLPENDRNYY
GCGEMPRHFAAKLDKYMYILPYKEFFGGVSGLTVEQFRKINGFPNAFWGWGGEDDDLWNR
VHYAGYNVTRPEGDLGKYKSIPHHHRGEVQFLGRYKLLRYSKERQYIDGLNNLIYRPKIL
VDRLYTNISVNLMPELAPIEDY
GenBank ID Protein 6521693
UniProtKB/Swiss-Prot ID Q9UBX8
UniProtKB/Swiss-Prot Entry Name B4GT6_HUMAN
PDB IDs Not Available
GenBank Gene ID AB024742
GeneCard ID B4GALT6
GenAtlas ID B4GALT6
HGNC ID HGNC:929
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Lo NW, Shaper JH, Pevsner J, Shaper NL: The expanding beta 4-galactosyltransferase gene family: messages from the databanks. Glycobiology. 1998 May;8(5):517-26. [PubMed:9597550 ]
  3. Amado M, Almeida R, Schwientek T, Clausen H: Identification and characterization of large galactosyltransferase gene families: galactosyltransferases for all functions. Biochim Biophys Acta. 1999 Dec 6;1473(1):35-53. [PubMed:10580128 ]
  4. Takizawa M, Nomura T, Wakisaka E, Yoshizuka N, Aoki J, Arai H, Inoue K, Hattori M, Matsuo N: cDNA cloning and expression of human lactosylceramide synthase. Biochim Biophys Acta. 1999 May 18;1438(2):301-4. [PubMed:10320813 ]