KEGG   PATHWAY: ko00600Help
Entry
ko00600                     Pathway                                

Name
Sphingolipid metabolism
Class
Metabolism; Lipid metabolism
BRITE hierarchy
Pathway map
ko00600  Sphingolipid metabolism
ko00600

Ortholog table
Module
M00066  Lactosylceramide biosynthesis [PATH:ko00600]
M00067  Sulfoglycolipids biosynthesis, ceramide/1-alkyl-2-acylglycerol => sulfatide/seminolipid [PATH:ko00600]
M00094  Ceramide biosynthesis [PATH:ko00600]
M00099  Sphingosine biosynthesis [PATH:ko00600]
M00100  Sphingosine degradation [PATH:ko00600]
Disease
H00125  Fabry disease
H00126  Gaucher disease
H00127  Metachromatic leukodystrophy
H00135  Krabbe disease
H00137  Niemann-Pick disease type A/B
H00138  Farber lipogranulomatosis
H00142  Sialidosis
H00265  Hereditary sensory and autonomic neuropathy
H00281  GM1 gangliosidosis
H00423  Sphingolipidosis
H00424  Defects in the degradation of sphingomyelin
H00426  Gangliosidosis
Other DBs
GO: 0006665 0030148
Orthology
K00654  serine palmitoyltransferase [EC:2.3.1.50]
K04708  3-dehydrosphinganine reductase [EC:1.1.1.102]
K04709  Acyl-CoA-dependent ceramide synthase [EC:2.3.1.24]
K04710  ceramide synthetase [EC:2.3.1.24]
K23727  sphingoid base N-palmitoyltransferase [EC:2.3.1.291]
K12348  acid ceramidase [EC:3.5.1.23]
K12349  neutral ceramidase [EC:3.5.1.23]
K01441  alkaline ceramidase [EC:3.5.1.23]
K04711  dihydroceramidase [EC:3.5.1.-]
K04712  sphingolipid 4-desaturase/C4-monooxygenase [EC:1.14.19.17 1.14.18.5]
K04713  sphinganine C4-monooxygenase [EC:1.14.18.5]
K05848  phytoceramidase [EC:3.5.1.-]
K04714  shingomyelin synthase [EC:2.7.8.27]
K12350  sphingomyelin phosphodiesterase [EC:3.1.4.12]
K12351  sphingomyelin phosphodiesterase 2 [EC:3.1.4.12]
K12352  sphingomyelin phosphodiesterase 3 [EC:3.1.4.12]
K12353  sphingomyelin phosphodiesterase 4 [EC:3.1.4.12]
K12354  ectonucleotide pyrophosphatase/phosphodiesterase family member 7 [EC:3.1.4.12]
K01117  sphingomyelin phosphodiesterase [EC:3.1.4.12]
K01123  sphingomyelin phosphodiesterase D [EC:3.1.4.41]
K04715  ceramide kinase [EC:2.7.1.138]
K01080  phosphatidate phosphatase [EC:3.1.3.4]
K04716  sphingosine-1-phosphate phosphatase 1 [EC:3.1.3.-]
K04717  sphingosine-1-phosphate phosphotase 2 [EC:3.1.3.-]
K23451  dihydrosphingosine 1-phosphate phosphatase [EC:3.1.3.-]
K04718  sphingosine kinase [EC:2.7.1.91]
K01634  sphinganine-1-phosphate aldolase [EC:4.1.2.27]
K00720  ceramide glucosyltransferase [EC:2.4.1.80]
K01201  glucosylceramidase [EC:3.2.1.45]
K17108  non-lysosomal glucosylceramidase [EC:3.2.1.45]
K07553  beta-1,4-galactosyltransferase 6 [EC:2.4.1.274]
K01190  beta-galactosidase [EC:3.2.1.23]
K12309  beta-galactosidase [EC:3.2.1.23]
K04628  ceramide galactosyltransferase [EC:2.4.1.47]
K01202  galactosylceramidase [EC:3.2.1.46]
K01019  galactosylceramide sulfotransferase [EC:2.8.2.11]
K01130  arylsulfatase [EC:3.1.6.1]
K01134  arylsulfatase A [EC:3.1.6.8]
K01186  sialidase-1 [EC:3.2.1.18]
K12357  sialidase-2/3/4 [EC:3.2.1.18]
K23550  sialidase [EC:3.2.1.18]
K01189  alpha-galactosidase [EC:3.2.1.22]
K07406  alpha-galactosidase [EC:3.2.1.22]
K07407  alpha-galactosidase [EC:3.2.1.22]
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
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
Reference
  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
  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
  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
  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
ko00260  Glycine, serine and threonine metabolism
ko00601  Glycosphingolipid biosynthesis - lacto and neolacto series
ko00603  Glycosphingolipid biosynthesis - globo and isoglobo series
ko00604  Glycosphingolipid biosynthesis - ganglio series
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