LPP1
label: PPAP2A
label: UniProt:O14494 PPAP2A

DEGS1
label: DES1
label: MIG15
label: MLD
label: UniProt:O15121 DEGS1

KIAA1252
label: SGPL1
label: UniProt:O95470 SGPL1

ASM
label: SMPD1
label: UniProt:P17405 SMPD1

GM2A
label: UniProt:P17900 GM2A

GALC
label: UniProt:P54803 GALC

CK1G2
label: CSNK1G2
label: UniProt:P78368 CSNK1G2

FVT1
label: KDSR
label: UniProt:Q06136 KDSR

PKD
label: PKD1
label: PRKCM
label: PRKD1
label: UniProt:Q15139 PRKD1

UGCG
label: UniProt:Q16739 UGCG

ACER2
label: ASAH3L
label: PP11646
label: UniProt:Q5QJU3 ACER2

C14orf66
label: DEGS2
label: UniProt:Q6QHC5 DEGS2

ENPP7
label: UNQ3077/PRO9912
label: UniProt:Q6UWV6 ENPP7

MOB
label: SGMS1
label: SMS1
label: TMEM23
label: UniProt:Q86VZ5 SGMS1

FGE
label: PSEC0152
label: SUMF1
label: UNQ3037/PRO9852
label: UniProt:Q8NBK3 SUMF1

SGMS2
label: SMS2
label: UniProt:Q8NHU3 SGMS2

CERK
label: KIAA1646
label: UniProt:Q8TCT0 CERK

ACER1
label: ASAH3
label: UniProt:Q8TDN7 ACER1

CST
label: GAL3ST1
label: UniProt:Q99999 GAL3ST1

CBG
label: CBGL1
label: GBA3
label: UniProt:Q9H227 GBA3

AD035
label: GBA2
label: KIAA1605
label: UniProt:Q9HCG7 GBA2

ASAH2
label: HNAC1
label: UniProt:Q9NR71 ASAH2

ACER3
label: APHC
label: PHCA
label: UniProt:Q9NUN7 ACER3

NEU3
label: UniProt:Q9UQ49 NEU3

NEU2
label: UniProt:Q9Y3R4 NEU2

SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
type: Restriction
  on property: enabled by
  some values from: FGE
type: Restriction
  on property: enabled by
  some values from: FGE
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005788
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005788
type: GO_0016491
BFO_0000050: Glycosphingolipid metabolism
directly activates: Active ARSA translocates to the lysosome

3-ketosphinganine + NADPH + H+ => sphinganine + NADP+
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: enabled by
  some values from: FVT1
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: enabled by
  some values from: FVT1
type: GO_0047560
BFO_0000050: Sphingolipid de novo biosynthesis

CERT + ATP => monophospho-CERT + ADP
type: Restriction
  on property: enabled by
  some values from: PKD
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: enabled by
  some values from: PKD
type: GO_0004674
BFO_0000050: Sphingolipid de novo biosynthesis

CERT [ER] + ceramide [ER] => CERT:ceramide [ER]
type: GO_0003674
BFO_0000050: Sphingolipid de novo biosynthesis
label: âCERTâ protein binds ceramide associated with the endoplasmic reticulum membrane

CERT:PPM1L:VAPA/B [ER] => CERT [cytosol] + PPM1L:VAPA/B [ER]
type: GO_0003674
BFO_0000050: Sphingolipid de novo biosynthesis
label: âCERTâ dissociates from the endoplasmic reticulum

CERT:ceramide [ER] => ceramide [Golgi] + CERT [ER]
type: GO_0003674
BFO_0000050: Sphingolipid de novo biosynthesis
directly activates: Ceramide glucosyltransferase (UGCG) catalyses the transfer of glucose to ceramide
directly activates: Catalysis2554
label: âCERTâ protein releases its bound ceramide into the membrane of the Golgi apparatus

ceramide + H2O <=> stearate + sphingosine [endoplasmic reticulum]
type: Restriction
  on property: enabled by
  some values from: ACER1
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: enabled by
  some values from: ACER1
type: GO_0017040
BFO_0000050: Sphingolipid de novo biosynthesis

ceramide + H2O => stearate + sphingosine [Golgi]
type: Restriction
  on property: enabled by
  some values from: ACER2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: Restriction
  on property: enabled by
  some values from: ACER2
type: GO_0017040
BFO_0000050: Sphingolipid de novo biosynthesis

dihydroceramide + NAD(P)H + H+ + O2 => ceramide + NAD(P)+ + H2O
type: Restriction
  on property: enabled by
  some values from: DEGS1
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: enabled by
  some values from: DEGS1
type: GO_0042284
BFO_0000050: Sphingolipid de novo biosynthesis

dihydroceramide + NADPH + H+ + O2 => phytoceramide + NADP+ + H2O
type: Restriction
  on property: enabled by
  some values from: C14orf66
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: enabled by
  some values from: C14orf66
type: GO_0000170
BFO_0000050: Sphingolipid de novo biosynthesis

monophospho-CERT + 2 ATP => multiphospho-CERT + 2 ADP
type: Restriction
  on property: enabled by
  some values from: CK1G2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: enabled by
  some values from: CK1G2
type: GO_0004674
BFO_0000050: Sphingolipid de novo biosynthesis

multiphospho-CERT + PPM1L:VAPA/B => multiphospho-CERT:PPM1L:VAPA/B
type: GO_0003674
BFO_0000050: Sphingolipid de novo biosynthesis

multiphospho-CERT:PPM1L:VAPA/B + 3 H2O => CERT:PPM1L:VAPA/B + 3 orthophosphate
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0004722
BFO_0000050: Sphingolipid de novo biosynthesis
directly activates: CERT:PPM1L:VAPA/B [ER] => CERT [cytosol] + PPM1L:VAPA/B [ER]

palmitoyl-CoA + serine => 3-ketosphinganine + CoASH + CO2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0004758
BFO_0000050: Sphingolipid de novo biosynthesis

phosphatidylcholine + ceramide <=> sphingomyelin + diacylglycerol [SGMS1]
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: Restriction
  on property: enabled by
  some values from: MOB
type: Restriction
  on property: enabled by
  some values from: MOB
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: GO_0033188
BFO_0000050: Sphingolipid de novo biosynthesis

phosphatidylcholine + ceramide <=> sphingomyelin + diacylglycerol [SGMS2]
type: Restriction
  on property: enabled by
  some values from: SGMS2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: enabled by
  some values from: SGMS2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0033188
BFO_0000050: Sphingolipid de novo biosynthesis

phytoceramide + H2O => stearate + phytosphingosine
type: Restriction
  on property: enabled by
  some values from: ACER3
type: Restriction
  on property: enabled by
  some values from: ACER3
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0070774
BFO_0000050: Sphingolipid de novo biosynthesis

sphinganine (dihydrosphingosine) +ATP => sphinganine 1-phosphate + ADP
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: GO_0008481
BFO_0000050: Sphingolipid de novo biosynthesis

sphinganine + stearyl-CoA => dihydroceramide + CoASH
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0050291
BFO_0000050: Sphingolipid de novo biosynthesis

sphinganine 1-phosphate + H2O => sphinganine + orthophosphate
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0070780
BFO_0000050: Sphingolipid de novo biosynthesis
directly activates: sphinganine (dihydrosphingosine) +ATP => sphinganine 1-phosphate + ADP
directly activates: Catalysis2524

sphinganine 1-phosphate => phosphoethanolamine + hexadecanal
type: Restriction
  on property: enabled by
  some values from: KIAA1252
type: Restriction
  on property: enabled by
  some values from: KIAA1252
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0008117
BFO_0000050: Sphingolipid de novo biosynthesis

sphingosine +ATP => sphingosine 1-phosphate + ADP
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: GO_0017050
BFO_0000050: Sphingolipid de novo biosynthesis

sphingosine 1-phosphate + H2O => sphingosine + orthophosphate [cytosolic - PPAP]
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0042392
BFO_0000050: Sphingolipid de novo biosynthesis
directly activates: sphingosine +ATP => sphingosine 1-phosphate + ADP
directly activates: Catalysis2528

sphingosine 1-phosphate + H2O => sphingosine + orthophosphate [cytosolic - SGPP]
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0042392
BFO_0000050: Sphingolipid de novo biosynthesis

sphingosine 1-phosphate + H2O => sphingosine + orthophosphate [extracellular]
type: Restriction
  on property: enabled by
  some values from: LPP1
type: Restriction
  on property: enabled by
  some values from: LPP1
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0042392
BFO_0000050: Sphingolipid de novo biosynthesis

sphingosine 1-phosphate => phosphoethanolamine + hexadec-2-enal
type: Restriction
  on property: enabled by
  some values from: KIAA1252
type: Restriction
  on property: enabled by
  some values from: KIAA1252
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0008117
BFO_0000050: Sphingolipid de novo biosynthesis

Beta-galactosidase hydrolyses GM1 to GM2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004565
BFO_0000050: Glycosphingolipid metabolism
directly activates: Ganglioside GM2 activator presents GM2 to hexosaminidase for cleavage
directly activates: Catalysis2534

Ganglioside GM2 activator presents GM2 to hexosaminidase for cleavage
type: Restriction
  on property: enabled by
  some values from: GM2A
type: Restriction
  on property: enabled by
  some values from: GM2A
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0030290
BFO_0000050: Glycosphingolipid metabolism
directly activates: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
directly activates: Catalysis2535

Hexosaminidase A cleaves GalNAc from GM2 to form GM3
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004563
BFO_0000050: Glycosphingolipid metabolism
directly activates: Ganglioside GM2 activator presents GM2 to hexosaminidase for cleavage
directly activates: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
directly activates: Catalysis2534
directly activates: Catalysis2536

Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004308
BFO_0000050: Glycosphingolipid metabolism
directly activates: Beta-galactosidase can also hydrolyse globosides to form cerebrosides
directly activates: Catalysis2537

Beta-galactosidase can also hydrolyse globosides to form cerebrosides
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004565
BFO_0000050: Glycosphingolipid metabolism

Both hexosaminidase A and B can cleave GalNAc from globoside
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004563
BFO_0000050: Glycosphingolipid metabolism
directly activates: Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers
directly activates: Catalysis2539

Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004557
BFO_0000050: Glycosphingolipid metabolism
directly activates: Beta-galactosidase can also hydrolyse globosides to form cerebrosides
directly activates: Catalysis2537

Active ARSA translocates to the lysosome
type: GO_0003674
BFO_0000050: Glycosphingolipid metabolism
directly activates: Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside
directly activates: Catalysis2540

Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004098
BFO_0000050: Glycosphingolipid metabolism
directly activates: Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide
directly activates: Catalysis2541

Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide
type: Restriction
  on property: enabled by
  some values from: GALC
type: Restriction
  on property: enabled by
  some values from: GALC
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004336
BFO_0000050: Glycosphingolipid metabolism
directly activates: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
directly activates: Catalysis2548

Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome)
type: Restriction
  on property: enabled by
  some values from: ASM
type: Restriction
  on property: enabled by
  some values from: ASM
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0004767
BFO_0000050: Glycosphingolipid metabolism
directly activates: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
directly activates: Catalysis2548

Sphingomyelin phosphodiesterase 2 and 3 (SMPD2 and 3) hydrolyse sphingomyelin to ceramide (plasma membrane)
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0004767
BFO_0000050: Glycosphingolipid metabolism
directly activates: Ceramide kinase (CERK) mediates the phosphorylation of ceramide
directly activates: Catalysis2555

Sphingomyelin phosphodiesterase 4 (SMPD4) hydrolyses sphingomyelin to ceramide (ER membrane)
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0004767
BFO_0000050: Glycosphingolipid metabolism

Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005765
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005765
type: GO_0004348
BFO_0000050: Glycosphingolipid metabolism
directly activates: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
directly activates: Catalysis2548

Glucosylceramidase 2 cleaves the glucosidic bond of glucocerebroside to form ceramide (plasma membrane)
type: Restriction
  on property: enabled by
  some values from: AD035
type: Restriction
  on property: enabled by
  some values from: AD035
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0004348
BFO_0000050: Glycosphingolipid metabolism

Glucosylceramidase 3 cleaves the glucosidic bond of glucocerebroside to form ceramide (cytosol)
type: Restriction
  on property: enabled by
  some values from: CBG
type: Restriction
  on property: enabled by
  some values from: CBG
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: GO_0004348
BFO_0000050: Glycosphingolipid metabolism

Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: Restriction
  on property: BFO_0000066
  some values from: GO_0043202
type: GO_0017040
BFO_0000050: Glycosphingolipid metabolism

Ectonucleotide pyrophosphatase/phosphodiesterase 7 (ENPP7) hydrolyses sphingomyelin
type: Restriction
  on property: enabled by
  some values from: ENPP7
type: Restriction
  on property: enabled by
  some values from: ENPP7
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0004767
BFO_0000050: Glycosphingolipid metabolism

Neu5Ac is cleaved from GM3 by NEU2 to form a globoside (cytosol)
type: Restriction
  on property: enabled by
  some values from: NEU2
type: Restriction
  on property: enabled by
  some values from: NEU2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005829
type: GO_0004308
BFO_0000050: Glycosphingolipid metabolism

Neu5Ac is cleaved from GM3 by NEU3 to form globoside (plasma membrane)
type: Restriction
  on property: enabled by
  some values from: NEU3
type: Restriction
  on property: enabled by
  some values from: NEU3
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0004308
BFO_0000050: Glycosphingolipid metabolism

Neutral ceramidase hydrolyses ceramide into sphingosine and free fatty acid (plasma membrane)
type: Restriction
  on property: enabled by
  some values from: ASAH2
type: Restriction
  on property: enabled by
  some values from: ASAH2
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0017040
BFO_0000050: Glycosphingolipid metabolism

Steryl sulfatase hydrolyses sulfate from steroid sulfates
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005789
type: GO_0004773
BFO_0000050: Glycosphingolipid metabolism

Ceramide glucosyltransferase (UGCG) catalyses the transfer of glucose to ceramide
type: Restriction
  on property: enabled by
  some values from: UGCG
type: Restriction
  on property: enabled by
  some values from: UGCG
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: GO_0008120
BFO_0000050: Glycosphingolipid metabolism

Ceramide kinase (CERK) mediates the phosphorylation of ceramide
type: Restriction
  on property: enabled by
  some values from: CERK
type: Restriction
  on property: enabled by
  some values from: CERK
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: Restriction
  on property: BFO_0000066
  some values from: GO_0005886
type: GO_0001729
BFO_0000050: Glycosphingolipid metabolism

Galactosylceramide sulfotransferase (GAL3ST1) mediates the sulfation of membrane glycolipids
type: Restriction
  on property: enabled by
  some values from: CST
type: Restriction
  on property: enabled by
  some values from: CST
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: Restriction
  on property: BFO_0000066
  some values from: GO_0000139
type: GO_0001733
BFO_0000050: Glycosphingolipid metabolism

Sphingolipid metabolism
type: GO_0008150

Sphingolipid de novo biosynthesis
type: GO_0008150
BFO_0000050: Sphingolipid metabolism

Glycosphingolipid metabolism
type: GO_0008150
BFO_0000050: Sphingolipid metabolism