KEGG   PATHWAY: hsa00600
Entry
hsa00600                    Pathway                                
Name
Sphingolipid metabolism - Homo sapiens (human)
Class
Metabolism; Lipid metabolism
Pathway map
hsa00600  Sphingolipid metabolism
hsa00600

Module
hsa_M00066  Lactosylceramide biosynthesis [PATH:hsa00600]
hsa_M00067  Sulfoglycolipids biosynthesis, ceramide/1-alkyl-2-acylglycerol => sulfatide/seminolipid [PATH:hsa00600]
hsa_M00094  Ceramide biosynthesis [PATH:hsa00600]
hsa_M00099  Sphingosine biosynthesis [PATH:hsa00600]
hsa_M00100  Sphingosine degradation [PATH:hsa00600]
Network
nt06014  Sphingolipid degradation
  Element
N00640  Hydrolysis of lactosylceramide
N00642  Saposin stimulation of GBA and GALC
N00644  Hydrolysis of galabiosylceramide
N00647  Hydrolysis of galactosylceramide sulfate
N00649  Hydrolysis of sphingomyelin
N01709  Hydrolysis of globoside
N01710  Hydrolysis of ganglioside
N01711  Hydrolysis of GA1
N01712  Hydrolysis of psychosine
Drug
D02784  Agalsidase alfa (USAN/INN)
D02810  Alglucerase (JAN/USAN/INN)
D03020  Imiglucerase (USAN/INN)
D03228  Agalsidase beta (INN)
D05031  Migalastat hydrochloride (JAN/USAN)
D05032  Miglustat (JAN/USAN/INN)
D09029  Velaglucerase alfa (USAN)
D09675  Taliglucerase alfa (USAN/INN)
D09893  Eliglustat (USAN/INN)
D09894  Eliglustat tartrate (JAN/USAN)
D10820  Olipudase alfa (USAN/INN)
D11439  Lucerastat (JAN/USAN/INN)
D11633  Venglustat (USAN/INN)
D11685  Pegunigalsidase alfa (USAN/INN)
D12462  Cebsulfase alfa (USAN/INN)
Other DBs
GO: 0006665
Organism
Homo sapiens (human) [GN:hsa]
Gene
10558  SPTLC1; serine palmitoyltransferase long chain base subunit 1 [KO:K00654] [EC:2.3.1.50]
10715  CERS1; ceramide synthase 1 [KO:K04710] [EC:2.3.1.299]
10825  NEU3; neuraminidase 3 [KO:K12357] [EC:3.2.1.18]
123099  DEGS2; delta 4-desaturase, sphingolipid 2 [KO:K04712] [EC:1.14.19.17 1.14.18.5]
125981  ACER1; alkaline ceramidase 1 [KO:K01441] [EC:3.5.1.23]
129807  NEU4; neuraminidase 4 [KO:K12357] [EC:3.2.1.18]
130367  SGPP2; sphingosine-1-phosphate phosphatase 2 [KO:K04717] [EC:3.1.3.-]
166929  SGMS2; sphingomyelin synthase 2 [KO:K04714] [EC:2.7.8.27 3.1.4.62]
204219  CERS3; ceramide synthase 3 [KO:K24622]
2531  KDSR; 3-ketodihydrosphingosine reductase [KO:K04708] [EC:1.1.1.102]
253782  CERS6; ceramide synthase 6 [KO:K23727]
2581  GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
2583  B4GALNT1; beta-1,4-N-acetyl-galactosaminyltransferase 1 [KO:K00725] [EC:2.4.1.92]
259230  SGMS1; sphingomyelin synthase 1 [KO:K04714] [EC:2.7.8.27 3.1.4.62]
2629  GBA1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
2717  GLA; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
2720  GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
2760  GM2A; ganglioside GM2 activator [KO:K12383]
29956  CERS2; ceramide synthase 2 [KO:K24621]
3073  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
3074  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
339221  ENPP7; ectonucleotide pyrophosphatase/phosphodiesterase 7 [KO:K12354] [EC:3.1.4.12]
340485  ACER2; alkaline ceramidase 2 [KO:K01441] [EC:3.5.1.23]
410  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
427  ASAH1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
4758  NEU1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
4759  NEU2; neuraminidase 2 [KO:K12357] [EC:3.2.1.18]
55304  SPTLC3; serine palmitoyltransferase long chain base subunit 3 [KO:K00654] [EC:2.3.1.50]
55331  ACER3; alkaline ceramidase 3 [KO:K04711] [EC:3.5.1.-]
55512  SMPD3; sphingomyelin phosphodiesterase 3 [KO:K12352] [EC:3.1.4.12]
55627  SMPD4; sphingomyelin phosphodiesterase 4 [KO:K12353] [EC:3.1.4.12]
5660  PSAP; prosaposin [KO:K12382]
56624  ASAH2; N-acylsphingosine amidohydrolase 2 [KO:K12349] [EC:3.5.1.23]
56848  SPHK2; sphingosine kinase 2 [KO:K04718] [EC:2.7.1.91]
57704  GBA2; glucosylceramidase beta 2 [KO:K17108] [EC:3.2.1.45]
64781  CERK; ceramide kinase [KO:K04715] [EC:2.7.1.138]
6609  SMPD1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
6610  SMPD2; sphingomyelin phosphodiesterase 2 [KO:K12351] [EC:3.1.4.12]
7357  UGCG; UDP-glucose ceramide glucosyltransferase [KO:K00720] [EC:2.4.1.80]
7368  UGT8; UDP glycosyltransferase 8 [KO:K04628] [EC:2.4.1.47]
768239  PSAPL1; prosaposin like 1 [KO:K12382]
79603  CERS4; ceramide synthase 4 [KO:K24621]
81537  SGPP1; sphingosine-1-phosphate phosphatase 1 [KO:K04716] [EC:3.1.3.-]
8560  DEGS1; delta 4-desaturase, sphingolipid 1 [KO:K04712] [EC:1.14.19.17 1.14.18.5]
8611  PLPP1; phospholipid phosphatase 1 [KO:K01080] [EC:3.1.3.4]
8612  PLPP2; phospholipid phosphatase 2 [KO:K01080] [EC:3.1.3.4]
8613  PLPP3; phospholipid phosphatase 3 [KO:K01080] [EC:3.1.3.4]
8877  SPHK1; sphingosine kinase 1 [KO:K04718] [EC:2.7.1.91]
8879  SGPL1; sphingosine-1-phosphate lyase 1 [KO:K01634] [EC:4.1.2.27]
91012  CERS5; ceramide synthase 5 [KO:K23727]
9331  B4GALT6; beta-1,4-galactosyltransferase 6 [KO:K07553] [EC:2.4.1.274]
9334  B4GALT5; beta-1,4-galactosyltransferase 5 [KO:K09905] [EC:2.4.1.274 2.4.1.-]
9514  GAL3ST1; galactose-3-O-sulfotransferase 1 [KO:K01019] [EC:2.8.2.11]
9517  SPTLC2; serine palmitoyltransferase long chain base subunit 2 [KO:K00654] [EC:2.3.1.50]
Compound
C00065  L-Serine
C00154  Palmitoyl-CoA
C00195  N-Acylsphingosine
C00319  Sphingosine
C00346  Ethanolamine phosphate
C00550  Sphingomyelin
C00836  Sphinganine
C01120  Sphinganine 1-phosphate
C01190  Glucosylceramide
C01290  Lactosylceramide
C01747  Psychosine
C02686  Galactosylceramide
C02934  3-Dehydrosphinganine
C02960  Ceramide 1-phosphate
C03108  D-Glucosylsphingosine
C03272  Globoside
C03405  Lactosylceramide sulfate
C03640  Sphingosyl-phosphocholine
C04730  GM3
C04737  alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
C04884  N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide
C04911  D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide
C05681  Ceramide 2-aminoethylphosphonate
C06062  Ceramide phosphoethanolamine
C06124  Sphingosine 1-phosphate
C06125  Sulfatide
C06126  Digalactosylceramide
C06127  Digalactosylceramide sulfate
C06128  GM4
C06135  GA2
C06136  GA1
C12126  Dihydroceramide
C12144  Phytosphingosine
C12145  Phytoceramide
C22467  Gangliotriaosylceramide-II3 sulfate
Reference
PMID:12011104
  Authors
Merrill AH Jr.
  Title
De novo sphingolipid biosynthesis: a necessary, but dangerous, pathway.
  Journal
J Biol Chem 277:25843-6 (2002)
DOI:10.1074/jbc.R200009200
Reference
PMID:10652340
  Authors
Tani M, Okino N, Mitsutake S, Tanigawa T, Izu H, Ito M.
  Title
Purification and characterization of a neutral ceramidase from mouse liver. A single protein catalyzes the reversible reaction in which ceramide is both hydrolyzed and synthesized.
  Journal
J Biol Chem 275:3462-8 (2000)
DOI:10.1074/jbc.275.5.3462
Reference
PMID:11305904
  Authors
Hannun YA, Luberto C, Argraves KM.
  Title
Enzymes of sphingolipid metabolism: from modular to integrative signaling.
  Journal
Biochemistry 40:4893-903 (2001)
DOI:10.1021/bi002836k
Reference
PMID:1495423
  Authors
Merrill AH Jr, Wang E.
  Title
Enzymes of ceramide biosynthesis.
  Journal
Methods Enzymol 209:427-37 (1992)
DOI:10.1016/0076-6879(92)09053-6
Reference
PMID:8457204
  Authors
Hirschberg K, Rodger J, Futerman AH.
  Title
The long-chain sphingoid base of sphingolipids is acylated at the cytosolic surface of the endoplasmic reticulum in rat liver.
  Journal
Biochem J 290 ( Pt 3):751-7 (1993)
DOI:10.1042/bj2900751
Reference
PMID:10366774
  Authors
Dickson RC, Lester RL.
  Title
Metabolism and selected functions of sphingolipids in the yeast Saccharomyces cerevisiae.
  Journal
Biochim Biophys Acta 1438:305-21 (1999)
DOI:10.1016/S1388-1981(99)00068-2
Related
pathway
hsa00260  Glycine, serine and threonine metabolism
hsa00601  Glycosphingolipid biosynthesis - lacto and neolacto series
hsa00603  Glycosphingolipid biosynthesis - globo and isoglobo series
hsa00604  Glycosphingolipid biosynthesis - ganglio series
KO pathway
ko00600   

  All links  
No link information was found.
KEGG   PATHWAY: hsa00600
Entry
hsa00600                    Pathway                                
Name
Sphingolipid metabolism - Homo sapiens (human)
Class
Metabolism; Lipid metabolism
Pathway map
hsa00600  Sphingolipid metabolism
hsa00600

Module
hsa_M00066  Lactosylceramide biosynthesis [PATH:hsa00600]
hsa_M00067  Sulfoglycolipids biosynthesis, ceramide/1-alkyl-2-acylglycerol => sulfatide/seminolipid [PATH:hsa00600]
hsa_M00094  Ceramide biosynthesis [PATH:hsa00600]
hsa_M00099  Sphingosine biosynthesis [PATH:hsa00600]
hsa_M00100  Sphingosine degradation [PATH:hsa00600]
Network
nt06014  Sphingolipid degradation
  Element
N00640  Hydrolysis of lactosylceramide
N00642  Saposin stimulation of GBA and GALC
N00644  Hydrolysis of galabiosylceramide
N00647  Hydrolysis of galactosylceramide sulfate
N00649  Hydrolysis of sphingomyelin
N01709  Hydrolysis of globoside
N01710  Hydrolysis of ganglioside
N01711  Hydrolysis of GA1
N01712  Hydrolysis of psychosine
Drug
D02784  Agalsidase alfa (USAN/INN)
D02810  Alglucerase (JAN/USAN/INN)
D03020  Imiglucerase (USAN/INN)
D03228  Agalsidase beta (INN)
D05031  Migalastat hydrochloride (JAN/USAN)
D05032  Miglustat (JAN/USAN/INN)
D09029  Velaglucerase alfa (USAN)
D09675  Taliglucerase alfa (USAN/INN)
D09893  Eliglustat (USAN/INN)
D09894  Eliglustat tartrate (JAN/USAN)
D10820  Olipudase alfa (USAN/INN)
D11439  Lucerastat (JAN/USAN/INN)
D11633  Venglustat (USAN/INN)
D11685  Pegunigalsidase alfa (USAN/INN)
D12462  Cebsulfase alfa (USAN/INN)
Other DBs
GO: 0006665
Organism
Homo sapiens (human) [GN:hsa]
Gene
10558  SPTLC1; serine palmitoyltransferase long chain base subunit 1 [KO:K00654] [EC:2.3.1.50]
10715  CERS1; ceramide synthase 1 [KO:K04710] [EC:2.3.1.299]
10825  NEU3; neuraminidase 3 [KO:K12357] [EC:3.2.1.18]
123099  DEGS2; delta 4-desaturase, sphingolipid 2 [KO:K04712] [EC:1.14.19.17 1.14.18.5]
125981  ACER1; alkaline ceramidase 1 [KO:K01441] [EC:3.5.1.23]
129807  NEU4; neuraminidase 4 [KO:K12357] [EC:3.2.1.18]
130367  SGPP2; sphingosine-1-phosphate phosphatase 2 [KO:K04717] [EC:3.1.3.-]
166929  SGMS2; sphingomyelin synthase 2 [KO:K04714] [EC:2.7.8.27 3.1.4.62]
204219  CERS3; ceramide synthase 3 [KO:K24622]
2531  KDSR; 3-ketodihydrosphingosine reductase [KO:K04708] [EC:1.1.1.102]
253782  CERS6; ceramide synthase 6 [KO:K23727]
2581  GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
2583  B4GALNT1; beta-1,4-N-acetyl-galactosaminyltransferase 1 [KO:K00725] [EC:2.4.1.92]
259230  SGMS1; sphingomyelin synthase 1 [KO:K04714] [EC:2.7.8.27 3.1.4.62]
2629  GBA1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
2717  GLA; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
2720  GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
2760  GM2A; ganglioside GM2 activator [KO:K12383]
29956  CERS2; ceramide synthase 2 [KO:K24621]
3073  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
3074  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
339221  ENPP7; ectonucleotide pyrophosphatase/phosphodiesterase 7 [KO:K12354] [EC:3.1.4.12]
340485  ACER2; alkaline ceramidase 2 [KO:K01441] [EC:3.5.1.23]
410  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
427  ASAH1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
4758  NEU1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
4759  NEU2; neuraminidase 2 [KO:K12357] [EC:3.2.1.18]
55304  SPTLC3; serine palmitoyltransferase long chain base subunit 3 [KO:K00654] [EC:2.3.1.50]
55331  ACER3; alkaline ceramidase 3 [KO:K04711] [EC:3.5.1.-]
55512  SMPD3; sphingomyelin phosphodiesterase 3 [KO:K12352] [EC:3.1.4.12]
55627  SMPD4; sphingomyelin phosphodiesterase 4 [KO:K12353] [EC:3.1.4.12]
5660  PSAP; prosaposin [KO:K12382]
56624  ASAH2; N-acylsphingosine amidohydrolase 2 [KO:K12349] [EC:3.5.1.23]
56848  SPHK2; sphingosine kinase 2 [KO:K04718] [EC:2.7.1.91]
57704  GBA2; glucosylceramidase beta 2 [KO:K17108] [EC:3.2.1.45]
64781  CERK; ceramide kinase [KO:K04715] [EC:2.7.1.138]
6609  SMPD1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
6610  SMPD2; sphingomyelin phosphodiesterase 2 [KO:K12351] [EC:3.1.4.12]
7357  UGCG; UDP-glucose ceramide glucosyltransferase [KO:K00720] [EC:2.4.1.80]
7368  UGT8; UDP glycosyltransferase 8 [KO:K04628] [EC:2.4.1.47]
768239  PSAPL1; prosaposin like 1 [KO:K12382]
79603  CERS4; ceramide synthase 4 [KO:K24621]
81537  SGPP1; sphingosine-1-phosphate phosphatase 1 [KO:K04716] [EC:3.1.3.-]
8560  DEGS1; delta 4-desaturase, sphingolipid 1 [KO:K04712] [EC:1.14.19.17 1.14.18.5]
8611  PLPP1; phospholipid phosphatase 1 [KO:K01080] [EC:3.1.3.4]
8612  PLPP2; phospholipid phosphatase 2 [KO:K01080] [EC:3.1.3.4]
8613  PLPP3; phospholipid phosphatase 3 [KO:K01080] [EC:3.1.3.4]
8877  SPHK1; sphingosine kinase 1 [KO:K04718] [EC:2.7.1.91]
8879  SGPL1; sphingosine-1-phosphate lyase 1 [KO:K01634] [EC:4.1.2.27]
91012  CERS5; ceramide synthase 5 [KO:K23727]
9331  B4GALT6; beta-1,4-galactosyltransferase 6 [KO:K07553] [EC:2.4.1.274]
9334  B4GALT5; beta-1,4-galactosyltransferase 5 [KO:K09905] [EC:2.4.1.274 2.4.1.-]
9514  GAL3ST1; galactose-3-O-sulfotransferase 1 [KO:K01019] [EC:2.8.2.11]
9517  SPTLC2; serine palmitoyltransferase long chain base subunit 2 [KO:K00654] [EC:2.3.1.50]
Compound
C00065  L-Serine
C00154  Palmitoyl-CoA
C00195  N-Acylsphingosine
C00319  Sphingosine
C00346  Ethanolamine phosphate
C00550  Sphingomyelin
C00836  Sphinganine
C01120  Sphinganine 1-phosphate
C01190  Glucosylceramide
C01290  Lactosylceramide
C01747  Psychosine
C02686  Galactosylceramide
C02934  3-Dehydrosphinganine
C02960  Ceramide 1-phosphate
C03108  D-Glucosylsphingosine
C03272  Globoside
C03405  Lactosylceramide sulfate
C03640  Sphingosyl-phosphocholine
C04730  GM3
C04737  alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
C04884  N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide
C04911  D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide
C05681  Ceramide 2-aminoethylphosphonate
C06062  Ceramide phosphoethanolamine
C06124  Sphingosine 1-phosphate
C06125  Sulfatide
C06126  Digalactosylceramide
C06127  Digalactosylceramide sulfate
C06128  GM4
C06135  GA2
C06136  GA1
C12126  Dihydroceramide
C12144  Phytosphingosine
C12145  Phytoceramide
C22467  Gangliotriaosylceramide-II3 sulfate
Reference
PMID:12011104
  Authors
Merrill AH Jr.
  Title
De novo sphingolipid biosynthesis: a necessary, but dangerous, pathway.
  Journal
J Biol Chem 277:25843-6 (2002)
DOI:10.1074/jbc.R200009200
Reference
PMID:10652340
  Authors
Tani M, Okino N, Mitsutake S, Tanigawa T, Izu H, Ito M.
  Title
Purification and characterization of a neutral ceramidase from mouse liver. A single protein catalyzes the reversible reaction in which ceramide is both hydrolyzed and synthesized.
  Journal
J Biol Chem 275:3462-8 (2000)
DOI:10.1074/jbc.275.5.3462
Reference
PMID:11305904
  Authors
Hannun YA, Luberto C, Argraves KM.
  Title
Enzymes of sphingolipid metabolism: from modular to integrative signaling.
  Journal
Biochemistry 40:4893-903 (2001)
DOI:10.1021/bi002836k
Reference
PMID:1495423
  Authors
Merrill AH Jr, Wang E.
  Title
Enzymes of ceramide biosynthesis.
  Journal
Methods Enzymol 209:427-37 (1992)
DOI:10.1016/0076-6879(92)09053-6
Reference
PMID:8457204
  Authors
Hirschberg K, Rodger J, Futerman AH.
  Title
The long-chain sphingoid base of sphingolipids is acylated at the cytosolic surface of the endoplasmic reticulum in rat liver.
  Journal
Biochem J 290 ( Pt 3):751-7 (1993)
DOI:10.1042/bj2900751
Reference
PMID:10366774
  Authors
Dickson RC, Lester RL.
  Title
Metabolism and selected functions of sphingolipids in the yeast Saccharomyces cerevisiae.
  Journal
Biochim Biophys Acta 1438:305-21 (1999)
DOI:10.1016/S1388-1981(99)00068-2
Related
pathway
hsa00260  Glycine, serine and threonine metabolism
hsa00601  Glycosphingolipid biosynthesis - lacto and neolacto series
hsa00603  Glycosphingolipid biosynthesis - globo and isoglobo series
hsa00604  Glycosphingolipid biosynthesis - ganglio series
KO pathway
ko00600   

  All links  
No link information was found.
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