KEGG   ENZYME: 2.4.1.47
Entry
EC 2.4.1.47                 Enzyme                                 

Name
N-acylsphingosine galactosyltransferase;
UGT8 (gene name);
CGT (gene name);
UDP galactose-N-acylsphingosine galactosyltransferase;
uridine diphosphogalactose-acylsphingosine galactosyltransferase;
UDP-galactose:N-acylsphingosine D-galactosyltransferase;
UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase;
2-hydroxyacylsphingosine 1-beta-galactosyltransferase
Class
Transferases;
Glycosyltransferases;
Hexosyltransferases
Sysname
UDP-alpha-D-galactose:N-acylsphingosine beta-D-galactosyltransferase (configuration-inverting)
Reaction(IUBMB)
UDP-alpha-D-galactose + a ceramide = UDP + a beta-D-galactosylceramide [RN:R01500 R06277]
Reaction(KEGG)
R01500 R06277(G) > R04322;
(other) R10804
Substrate
UDP-alpha-D-galactose [CPD:C00052];
ceramide [CPD:C00195]
Product
UDP [CPD:C00015];
beta-D-galactosylceramide
Comment
This membrane-bound, endoplasmic reticulum-located enzyme catalyses the last step in the synthesis of galactocerebrosides, which are abundant sphingolipids of the myelin membrane of the central nervous system and peripheral nervous system. It has a strong preference for ceramides that contain hydroxylated fatty acids.
History
EC 2.4.1.47 created 1972
Pathway
ec00565  Ether lipid metabolism
ec00600  Sphingolipid metabolism
ec01100  Metabolic pathways
Orthology
K04628  ceramide galactosyltransferase
Genes
HSA: 7368(UGT8)
PTR: 461446(UGT8)
PPS: 100985864(UGT8)
GGO: 101137553(UGT8)
PON: 100437492(UGT8)
NLE: 100607960(UGT8)
MCC: 702979(UGT8)
MCF: 102126400(UGT8)
CSAB: 103236153(UGT8)
RRO: 104675972(UGT8)
RBB: 108527094(UGT8)
CJC: 100396627(UGT8)
SBQ: 101036737(UGT8)
MMU: 22239(Ugt8a)
MCAL: 110291396(Ugt8)
MPAH: 110320435(Ugt8)
RNO: 50555(Ugt8)
MUN: 110554355
CGE: 100772408
NGI: 103731259(Ugt8)
HGL: 101702896(Ugt8)
CCAN: 109677276(Ugt8)
OCU: 100341450(UGT8)
TUP: 102480796(UGT8)
CFA: 487910(UGT8)
VVP: 112930967(UGT8)
AML: 100465291(UGT8)
UMR: 103661766(UGT8)
UAH: 113255592(UGT8)
ORO: 101383800(UGT8)
ELK: 111156987
FCA: 101097722(UGT8)
PTG: 102965984(UGT8)
PPAD: 109248503(UGT8)
AJU: 106977315(UGT8)
BTA: 281566(UGT8)
BOM: 102269259(UGT8)
BIU: 109560234(UGT8)
BBUB: 102409846(UGT8)
CHX: 102174638(UGT8)
OAS: 101113069(UGT8)
SSC: 100511825(UGT8)
CFR: 102522568(UGT8)
CDK: 105089918(UGT8)
BACU: 103004850(UGT8)
LVE: 103073438(UGT8)
OOR: 101289765(UGT8)
DLE: 111186063(UGT8)
PCAD: 102993924(UGT8)
ECB: 100072587(UGT8)
EPZ: 103557103(UGT8)
EAI: 106842868(UGT8)
MYB: 102252842(UGT8)
MNA: 107527850(UGT8)
HAI: 109390271(UGT8)
DRO: 112312608(UGT8)
PALE: 102881397(UGT8)
RAY: 107510863(UGT8)
MJV: 108409879(UGT8)
LAV: 100665032(UGT8)
TMU: 101352658
MDO: 100010696(UGT8)
SHR: 100927545(UGT8)
PCW: 110218601(UGT8)
OAA: 100076336(UGT8)
GGA: 374033(UGT8) 428949(UGT8L)
MGP: 100539821 100541199(UGT8)
CJO: 107313583(UGT8) 107315928
NMEL: 110399157(UGT8) 110400413
APLA: 101794817 101799126(UGT8)
ACYG: 106029878 106038627(UGT8)
TGU: 100228968 100231902(UGT8)
LSR: 110471176(UGT8) 110480032
SCAN: 103813974 103815551(UGT8)
GFR: 102031520(UGT8) 102040819
FAB: 101807254 101809664(UGT8)
PHI: 102107687(UGT8) 102108262
PMAJ: 107203102(UGT8) 107206597
CCAE: 111928999(UGT8) 111931100
CCW: 104696845(UGT8) 104697059
ETL: 114054721 114068337(UGT8)
FPG: 101914948(UGT8) 101924669
FCH: 102047748 102051681(UGT8)
CLV: 102089904(UGT8) 102097199
NNI: 104019136(UGT8) 104019447
ACUN: 113479767(UGT8) 113481588
PADL: 103915563 103926098(UGT8)
AAM: 106492128(UGT8) 106498896
ASN: 102382334(UGT8) 102384980
AMJ: 102574992 106737104(UGT8)
CMY: 102936934(UGT8) 102941238
CPIC: 101945157 101952125(UGT8)
ACS: 100551810(ugt8)
PVT: 110083901(UGT8)
PBI: 103055483(UGT8)
PMUR: 107296372(UGT8)
TSR: 106556413(UGT8)
GJA: 107110289(UGT8) 107122753
XLA: 108706712(ugt8.S) 108710441(ugt8.L)
XTR: 779534(ugt8)
NPR: 108792209(UGT8)
DRE: 553508(ugt8)
SGH: 107548345(ugt8) 107591551
IPU: 108260842(ugt8)
PHYP: 113544363(ugt8)
AMEX: 103043793(ugt8)
EEE: 113586472(ugt8)
TRU: 101064956(ugt8)
LCO: 104929353(ugt8)
NCC: 104955311(ugt8)
MZE: 101473219(ugt8)
ONL: 100696128(ugt8)
OLA: 101156303(ugt8)
XMA: 102221616(ugt8)
XCO: 114144945(ugt8)
PRET: 103467322(ugt8)
CVG: 107085760(ugt8)
NFU: 107375211(ugt8)
KMR: 108231012(ugt8)
ALIM: 106517139(ugt8)
AOCE: 111568569(ugt8)
CSEM: 103383812(ugt8)
POV: 109637829(ugt8)
LCF: 108896622(ugt8)
SDU: 111231488(ugt8)
SLAL: 111664066(ugt8)
HCQ: 109519902(ugt8)
BPEC: 110165331(ugt8)
MALB: 109961392(ugt8)
ELS: 105009093(ugt8)
SFM: 108924325(ugt8)
PKI: 111837341(ugt8)
LCM: 102350700 102364207(UGT8)
BFO: 118417462
DER: 6541458
DSI: Dsimw501_GD10375(Dsim_GD10375)
DPE: 6590768
DMN: 108160067
DAZ: 108614624
DNV: 108651678
DHE: 111605641
MDE: 101892333
AALB: 115263973
AME: 411298
BIM: 100744553
OBB: 114872868
ACEP: 105619733
CFO: 105255889
LHU: 105673022
PGC: 109853431
OBO: 105284682
DPA: 109536734
BMOR: 100862844(UGT50A1)
BMAN: 114244370
PMAC: 106719871
TNL: 113504541
BTAB: 109038550
FCD: 110844391
PVM: 113821451
CBR: CBG01383
OBI: 106870503
PDAM: 113676364
APRO: F751_1121
 » show all
Reference
1  [PMID:5807218]
  Authors
Fujino Y, Nakano M.
  Title
Enzymic synthesis of cerebroside from ceramide and uridine diphosphate galactose.
  Journal
Biochem J 113:573-5 (1969)
DOI:10.1042/bj1130573
Reference
2  [PMID:5793706]
  Authors
Morell P, Radin NS.
  Title
Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing hydroxy fatty acid.
  Journal
Biochemistry 8:506-12 (1969)
DOI:10.1021/bi00830a008
Reference
3  [PMID:5497154]
  Authors
Morell P, Costantino-Ceccarini E, Radin NS
  Title
The biosynthesis by brain microsomes of cerebrosides containing nonhydroxy fatty  acids.
  Journal
Arch Biochem Biophys 141:738-48 (1970)
DOI:10.1016/0003-9861(70)90192-X
Reference
4  [PMID:5090043]
  Authors
Basu S, Schultz AM, Basu M, Roseman S.
  Title
Enzymatic synthesis of galactocerebroside by a galactosyltransferase from embryonic chicken brain.
  Journal
J Biol Chem 246:4272-9 (1971)
Reference
5  [PMID:762114]
  Authors
Akanuma H, Kishimoto Y
  Title
Synthesis of ceramides and cerebrosides containing both alpha-hydroxy and nonhydroxy fatty acids from lignoceroyl-CoA by rat brain microsomes.
  Journal
J Biol Chem 254:1050-60 (1979)
Reference
6  [PMID:7306007]
  Authors
Koul O, Jungalwala FB
  Title
UDP-galactose:ceramide galactosyltransferase of rat central-nervous-system myelin.
  Journal
Biochem J 194:633-7 (1981)
DOI:10.1042/bj1940633
Reference
7  [PMID:7694285]
  Authors
Schulte S, Stoffel W
  Title
Ceramide UDPgalactosyltransferase from myelinating rat brain: purification, cloning, and expression.
  Journal
Proc Natl Acad Sci U S A 90:10265-9 (1993)
DOI:10.1073/pnas.90.21.10265
  Sequence
[rno:50555]
Reference
8  [PMID:9748263]
  Authors
Sprong H, Kruithof B, Leijendekker R, Slot JW, van Meer G, van der Sluijs P
  Title
UDP-galactose:ceramide galactosyltransferase is a class I integral membrane protein of the endoplasmic reticulum.
  Journal
J Biol Chem 273:25880-8 (1998)
DOI:10.1074/jbc.273.40.25880
Reference
9  [PMID:15998297]
  Authors
Fewou SN, Bussow H, Schaeren-Wiemers N, Vanier MT, Macklin WB, Gieselmann V, Eckhardt M
  Title
Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase.
  Journal
J Neurochem 94:469-81 (2005)
DOI:10.1111/j.1471-4159.2005.03221.x
Other DBs
ExplorEnz - The Enzyme Database: 2.4.1.47
IUBMB Enzyme Nomenclature: 2.4.1.47
ExPASy - ENZYME nomenclature database: 2.4.1.47
BRENDA, the Enzyme Database: 2.4.1.47
CAS: 37277-56-8
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