ENTRY       hsa00600                    Pathway
NAME        Sphingolipid metabolism - Homo sapiens (human)
CLASS       Metabolism; Lipid metabolism
PATHWAY_MAP hsa00600  Sphingolipid metabolism
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
DRUG        D02784  Agalsidase alfa (USAN/INN)
            D03228  Agalsidase beta (INN)
            D05031  Migalastat hydrochloride (JAN/USAN)
            D05032  Miglustat (JAN/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)
            D12462  Cebsulfase alfa (USAN)
DBLINKS     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]
            9517  SPTLC2; serine palmitoyltransferase long chain base subunit 2 [KO:K00654] [EC:2.3.1.50]
            55304  SPTLC3; serine palmitoyltransferase long chain base subunit 3 [KO:K00654] [EC:2.3.1.50]
            2531  KDSR; 3-ketodihydrosphingosine reductase [KO:K04708] [EC:1.1.1.102]
            10715  CERS1; ceramide synthase 1 [KO:K04710] [EC:2.3.1.299]
            29956  CERS2; ceramide synthase 2 [KO:K24621] [EC:2.3.1.297]
            79603  CERS4; ceramide synthase 4 [KO:K24621] [EC:2.3.1.297]
            204219  CERS3; ceramide synthase 3 [KO:K24622] [EC:2.3.1.297 2.3.1.298]
            253782  CERS6; ceramide synthase 6 [KO:K23727] [EC:2.3.1.291]
            91012  CERS5; ceramide synthase 5 [KO:K23727] [EC:2.3.1.291]
            427  ASAH1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
            56624  ASAH2; N-acylsphingosine amidohydrolase 2 [KO:K12349] [EC:3.5.1.23]
            340485  ACER2; alkaline ceramidase 2 [KO:K01441] [EC:3.5.1.23]
            125981  ACER1; alkaline ceramidase 1 [KO:K01441] [EC:3.5.1.23]
            55331  ACER3; alkaline ceramidase 3 [KO:K04711] [EC:3.5.1.-]
            8560  DEGS1; delta 4-desaturase, sphingolipid 1 [KO:K04712] [EC:1.14.19.17 1.14.18.5]
            123099  DEGS2; delta 4-desaturase, sphingolipid 2 [KO:K04712] [EC:1.14.19.17 1.14.18.5]
            259230  SGMS1; sphingomyelin synthase 1 [KO:K04714] [EC:2.7.8.27]
            166929  SGMS2; sphingomyelin synthase 2 [KO:K04714] [EC:2.7.8.27]
            6609  SMPD1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
            6610  SMPD2; sphingomyelin phosphodiesterase 2 [KO:K12351] [EC:3.1.4.12]
            55512  SMPD3; sphingomyelin phosphodiesterase 3 [KO:K12352] [EC:3.1.4.12]
            55627  SMPD4; sphingomyelin phosphodiesterase 4 [KO:K12353] [EC:3.1.4.12]
            339221  ENPP7; ectonucleotide pyrophosphatase/phosphodiesterase 7 [KO:K12354] [EC:3.1.4.12]
            64781  CERK; ceramide kinase [KO:K04715] [EC:2.7.1.138]
            8611  PLPP1; phospholipid phosphatase 1 [KO:K01080] [EC:3.1.3.4]
            8613  PLPP3; phospholipid phosphatase 3 [KO:K01080] [EC:3.1.3.4]
            8612  PLPP2; phospholipid phosphatase 2 [KO:K01080] [EC:3.1.3.4]
            81537  SGPP1; sphingosine-1-phosphate phosphatase 1 [KO:K04716] [EC:3.1.3.-]
            130367  SGPP2; sphingosine-1-phosphate phosphatase 2 [KO:K04717] [EC:3.1.3.-]
            8877  SPHK1; sphingosine kinase 1 [KO:K04718] [EC:2.7.1.91]
            56848  SPHK2; sphingosine kinase 2 [KO:K04718] [EC:2.7.1.91]
            8879  SGPL1; sphingosine-1-phosphate lyase 1 [KO:K01634] [EC:4.1.2.27]
            7357  UGCG; UDP-glucose ceramide glucosyltransferase [KO:K00720] [EC:2.4.1.80]
            2629  GBA1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
            57704  GBA2; glucosylceramidase beta 2 [KO:K17108] [EC:3.2.1.45]
            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.-]
            2720  GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
            7368  UGT8; UDP glycosyltransferase 8 [KO:K04628] [EC:2.4.1.47]
            2581  GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
            9514  GAL3ST1; galactose-3-O-sulfotransferase 1 [KO:K01019] [EC:2.8.2.11]
            410  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
            4758  NEU1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
            10825  NEU3; neuraminidase 3 [KO:K12357] [EC:3.2.1.18]
            129807  NEU4; neuraminidase 4 [KO:K12357] [EC:3.2.1.18]
            4759  NEU2; neuraminidase 2 [KO:K12357] [EC:3.2.1.18]
            2717  GLA; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
            2583  B4GALNT1; beta-1,4-N-acetyl-galactosaminyltransferase 1 [KO:K00725] [EC:2.4.1.92]
            3073  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
            3074  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
            5660  PSAP; prosaposin [KO:K12382]
            768239  PSAPL1; prosaposin like 1 [KO:K12382]
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
            C03405  Lactosylceramide sulfate
            C03640  Sphingosyl-phosphocholine
            C05681  Ceramide 2-aminoethylphosphonate
            C06062  Ceramide phosphoethanolamine
            C06124  Sphingosine 1-phosphate
            C06125  Sulfatide
            C06126  Digalactosylceramide
            C06127  Digalactosylceramide sulfate
            C06128  GM4
            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
REL_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
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