KEGG   PATHWAY: hsa00600Help
Entry
hsa00600                    Pathway                                

Name
Sphingolipid metabolism - Homo sapiens (human)
Class
Metabolism; Lipid metabolism
BRITE hierarchy
Pathway map
hsa00600  Sphingolipid metabolism
hsa00600

Ortholog table
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
N00641  GBA deficiency in lactosylceramide hydrolysis
N00644  Hydrolysis of galabiosylceramide
N00645  GLA deficiency in galabiosylceramide hydrolysis
N00646  GALC deficiency in galactosylceramide hydrolysis
N00647  Hydrolysis of galactosylceramide sulfate
N00648  ARSA deficiency in galactosylceramide sulfate hydrolysis
N00649  Hydrolysis of sphingomyelin
N00650  SMPD1 deficiency in sphingolipid degradation
N00651  ASAH1 deficiency in sphingolipid degradation
N00652  GLA mutation
N00679  Glucosylceramide synthesis in GBA deficiency
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
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)
D11439  Lucerastat (USAN/INN)
D11633  Venglustat (USAN/INN)
Other DBs
GO: 0006665 0030148
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]
10715  CERS1; ceramide synthase 1 [KO:K04710] [EC:2.3.1.24]
79603  CERS4; ceramide synthase 4 [KO:K04710] [EC:2.3.1.24]
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:1.14.18.5 1.14.19.17]
123099  DEGS2; delta 4-desaturase, sphingolipid 2 [KO:K04712] [EC:1.14.18.5 1.14.19.17]
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  GBA; glucosylceramidase beta [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]
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]
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
KO pathway
ko00600   
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