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) typeA and B
H00138  
Farber lipogranulomatosis
H00142  
Sialidosis
H00265  
Hereditary sensory and autonomic neuropathy (HSAN)
H00276  
Galactosialidosis
H00281  
GM1 gangliosidosis
H00423  
Defects in the degradation of sulfatide
H00424  
Defects in the degradation of sphingomyelin
H00426  
Defects in the degradation of ganglioside
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]
57704  
GBA2; glucosidase, beta (bile acid) 2 [KO:K17108] [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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