KEGG   PATHWAY: hsa00600Help
Entry
hsa00600                    Pathway                                
Name
Sphingolipid metabolism - Homo sapiens (human)
Class
Metabolism; Lipid metabolism
BRITE hierarchy
Pathway map
Sphingolipid metabolism
hsa00600

All organismsOrtholog table
Module
Lactosylceramide biosynthesis [PATH:hsa00600]
Cerebroside and sulfatide biosynthesis [PATH:hsa00600]
Ceramide biosynthesis [PATH:hsa00600]
Sphingosine biosynthesis [PATH:hsa00600]
Sphingosine degradation [PATH:hsa00600]
Disease
H00125  
Fabry disease
H00126  
Gaucher disease
H00127  
Metachromatic leukodystrophy (MLD)
H00135  
Krabbe disease
H00137  
Niemann-Pick disease (NPD), including;
H00138  
Farber lipogranulomatosis
H00142  
Sialidosis
H00276  
Galactosialidosis
H00281  
GM1 gangliosidosis
H00810  
Progressive myoclonic epilepsy (PME)
Drug
D05032  
Miglustat (JAN/USAN/INN)
D09893  
Eliglustat (USAN/INN)
D09894  
Eliglustat tartrate (USAN)
Other DBs
BSID: 
GO: 
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]
29956  
CERS2; ceramide synthase 2 [KO:K04710] [EC:2.3.1.24]
204219  
CERS3; ceramide synthase 3 [KO:K04710] [EC:2.3.1.24]
253782  
CERS6; ceramide synthase 6 [KO:K04710] [EC:2.3.1.24]
10715  
CERS1; ceramide synthase 1 [KO:K04710] [EC:2.3.1.24]
79603  
CERS4; ceramide synthase 4 [KO:K04710] [EC:2.3.1.24]
91012  
CERS5; ceramide synthase 5 [KO:K04710] [EC:2.3.1.24]
427  
ASAH1; N-acylsphingosine amidohydrolase (acid ceramidase) 1 [KO:K12348] [EC:3.5.1.23]
56624  
ASAH2; N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 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.-.-]
123099  
DEGS2; delta(4)-desaturase, sphingolipid 2 [KO:K04712] [EC:1.14.-.-]
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, acid lysosomal [KO:K12350] [EC:3.1.4.12]
6610  
SMPD2; sphingomyelin phosphodiesterase 2, neutral membrane (neutral sphingomyelinase) [KO:K12351] [EC:3.1.4.12]
55512  
SMPD3; sphingomyelin phosphodiesterase 3, neutral membrane (neutral sphingomyelinase II) [KO:K12352] [EC:3.1.4.12]
55627  
SMPD4; sphingomyelin phosphodiesterase 4, neutral membrane (neutral sphingomyelinase-3) [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  
PPAP2A; phosphatidic acid phosphatase type 2A [KO:K01080] [EC:3.1.3.4]
8613  
PPAP2B; phosphatidic acid phosphatase type 2B [KO:K01080] [EC:3.1.3.4]
8612  
PPAP2C; phosphatidic acid phosphatase type 2C [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  
GBA; glucosidase, beta, acid [KO:K01201] [EC:3.2.1.45]
9331  
B4GALT6; UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 6 [KO:K07553] [EC:2.4.1.274]
2720  
GLB1; galactosidase, beta 1 [KO:K12309] [EC:3.2.1.23]
7368  
UGT8; UDP glycosyltransferase 8 [KO:K04628] [EC:2.4.1.45]
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; sialidase 1 (lysosomal sialidase) [KO:K01186] [EC:3.2.1.18]
10825  
NEU3; sialidase 3 (membrane sialidase) [KO:K12357] [EC:3.2.1.18]
129807  
NEU4; sialidase 4 [KO:K12357] [EC:3.2.1.18]
4759  
NEU2; sialidase 2 (cytosolic sialidase) [KO:K12357] [EC:3.2.1.18]
2717  
GLA; galactosidase, alpha [KO:K01189] [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  
beta-D-Glucosyl-(1<->1)-ceramide
C01290  
beta-D-Galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
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  
Digalactosylceramidesulfate
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)
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)
Reference
  Authors
Hannun YA, Luberto C, Argraves KM.
  Title
Enzymes of sphingolipid metabolism: from modular to integrative signaling.
  Journal
Biochemistry 40:4893-903 (2001)
Reference
PMID:1495423
  Authors
Merrill AH Jr, Wang E.
  Title
Enzymes of ceramide biosynthesis.
  Journal
Methods Enzymol 209:427-37 (1992)
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)
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)
KO pathway
 
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