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Sphingolipid metabolism
Ceramide-centered synthesis and turnover of sphingomyelins, glycosphingolipids and signaling sphingoid phosphates. 77 reactions, from Reactome R-HSA-428157.
- GBA1:SAPC hydrolyzes GlcCerGlcCer + H2O → CERA + Glc
- bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3GM2A:GM2 + H2O → GalNAc + GM3 + GM2A(32-193)
- Beta-galactosidases hydrolyse mobilized GM1 to mobilized GM2H2O + mobilized GM1 → Gal + mobilized GM2
- bHEXA,bHEXB hydrolyze PSAP(195-273):Gb4Cer:PEH2O + PSAP(195-273):Gb4Cer:PE → GalNAc + PSAP(195-273):Gb3Cer:PE
- GM2A binds and mobilizes ligandsGM2A(32-193) + GM2A ligands → GM2A:Ligands
- NEU2 cleaves GM3 to form LacCer (cytosol)GM3 + H2O → Neu5Ac + LacCer
- NEU1,4 hydrolyze PSAP(195-273):GM3:PEH2O + PSAP(195-273):GM3:PE → Neu5Ac + PSAP(195-273):LacCer:PE
- GLA hydrolyzes PSAP(195-273):Gb3Cer:PEH2O + PSAP(195-273):Gb3Cer:PE → Gal + PSAP(195-273):LacCer:PE
- NEU3 cleaves GM3 to form LacCer (plasma membrane)GM3 + H2O → Neu5Ac + LacCer
- SMPD1 hydrolyzes SPHMSPHM d18:1 + H2O → CERA + ChoP + H+
- SMPD2 and 3 hydrolyze sphingomyelin (plasma membrane)SPHM d18:1 + H2O → CERA + ChoP + H+
- SMPD4 hydrolyzes sphingomyelin (ER membrane)SPHM d18:1 + H2O → CERA + ChoP + H+
- GLB1 hydrolyzes SapB/C:LacCerH2O + Sap B/C:LacCer → Gal + Sap B/C:GlcCer
- GALC hydrolyzes GalCerH2O + GalCer → CERA + Glc
- ASAH2 hydrolyzes ceramide (plasma membrane)CERA + H2O → SPG + RCOO-
- ASAH1 hydrolyzes ceramideCERA + H2O → SPG + RCOO-
- ARSA removes sulfate from PSAP(195-273):SulfatideH2O + PSAP(195-273):Sulfatide:PE → GalCer + SO4(2-) + PSAP(195-273) dimer + PE 30,32,34 + H+
- SUMF2 binds SUMF1, inhibiting SUMF1-mediated activation of arylsulfatasesSUMF1 + SUMF2 → SUMF1:SUMF2
- SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatasesARS + NAD+ → active ARS + NADH + H2S + H+
- UGCG transfers glucose to ceramideUDP-Glc + CERA-d18 → GlcCer + UDP + H+
- CERK phosphorylates CERA to form C1PATP + CERA → ADP + C1P + H+
- ENPP7 hydrolyzes sphingomyelinH2O + SPHM d18:1 → CERA + ChoP + H+
- GBA2 hydrolyzes GlcCer (plasma membrane)GlcCer + H2O → CERA + Glc
- GBA3 hydrolyzes GlcCer (cytosol)GlcCer + H2O → CERA + Glc
- M6PR transports activated ARSA to the lysosomeactive ARSA:Ca2+ → active ARSA:Ca2+
- KDSR reduces 3-ketosphingoidH+ + NADPH + 3-ketosphingoid → NADP+ + sphingoid base
- SPTLC complexes transfer acyl-CoA onto serineL-Ser + acyl-CoA + H+ → CoA-SH + CO2 + 3-ketosphingoid
- Ceramide synthases transfer acyl-CoA onto sphingoidacyl-CoA + sphingoid base → DHCE + CoA-SH + H+
- ACER2 hydrolyzes ceramide (Golgi)H2O + CERA → RCOO- + SPG
- ACER1 hydrolyzes ceramide (endoplasmic reticulum)H2O + CERA → RCOO- + SPG
- DEGS1 dehydrogenates dihydroceramide2 H+ + O2 + DHCE + 2 CYB5B:Fe2+ → 2 H2O + (4E)-CERA + 2 CYB5B:Fe3+
- DEGS2 oxygenates dihydroceramideH+ + O2 + DHCE + CYB5B:Fe2+ → H2O + phytoceramide + CYB5B:Fe3+
- ACER3 hydrolyzes phytoceramideH2O + phytoceramide-C16 → phytosphingosine + RCOO-
- SPHK1 phosphorylates sphingoidATP + SPG → ADP + S1P + H+
- SGPP1,2 dephosphorylate sphingoid-1-phosphatesH2O + S1P → Pi + SPG
- PXLP-SGPL1 cleaves sphingoid-1-phosphatesS1P,SPAP → PETA + HXAL,HD2NAL
- PLPP1 dephosphorylates extracellular sphingosine-1-phosphateH2O + C18-S1P → Pi + sphingosine
- PLPP1,2,3 dephosphorylate cytosolic sphingosine-1-phosphateH2O + C18-S1P → Pi + sphingosine
- PRKD1,2,3 phosphorylates CERT1-2ATP + CERT1-2 → ADP + p-S132-CERT1-2 + H+
- CSNK1G2 phosphorylates p-CERT1-22 ATP + p-S132-CERT1-2 → 2 ADP + p-S,S132,T-CERT1-2 + 2 H+
- PPM1L dephosphorylates multiphospho-CERT1-23 H2O + PPM1L:VAPA/B dimer + p-S,S132,T-CERT1-2 → CERT1-2:PPM1L:VAPA/B dimer + 3 Pi
- SGMS2 transfers phosphocholine onto ceramidePC + CERA → SPHM + DAG
- SGMS1 transfers phosphocholine onto ceramidePC + CERA → SPHM + DAG
- ALDH3A2-1 oxidises HD2NAL to PALMH2O + HD2NAL + NAD+ → H+ + NADH + PALM
- FA2H hydroxylates 1,2-saturated fatty acidsO2 + 2-saturated fatty acid + 2 CYB5B:Fe2+ + 2 H+ → H2O + 2-OH FA + 2 CYB5B:Fe3+
- ALDH3B1 oxidises HXAL to PALMHXAL + H2O + NAD+ → PALM + NADH + H+
- UGT8 transfers Gal from UDP-Gal to CERACERA + UDP-Gal → GalCer + UDP + H+
- ALDH3B2 oxidises HXAL to PALMHXAL + H2O + NAD+ → PALM + NADH + H+
- B4GALNT1 dimer transfers GalNAc to gangliosidesGD3,GM3,GT3,SM3,LacCer + UDP-GalNAc → GD2,GM2, GA2,GT2,SM2 + UDP + H+
- B3GALNT1 transfers GalNAc to Gb3Cer to form Gb4CerGb3Cer + UDP-GalNAc → Gb4Cer + UDP + H+
- SAMD8 transfers phosphatidyl from PE onto C16DH CERPE + C16DH CER → CPE + DAG
- SPNS2,MFSD2B transport S1P from cytosol to extracellular regionS1P → S1P
- SPHK2 binds SPHK2 inhibitorsSPHK2 + SPHK2 inhibitors → SPHK2:SPHK2 inhibitors
- SPHK2 phosphorylates sphingoidATP + SPG → ADP + H+ + S1P
- PSAP is cleavedPSAP → PSAP(195-273) + PSAP(311-391) + PSAP(405-486) + PSAP(60-142)
- PSAP(195-273) dimer:PE binds and mobilizes ligandsPSAP(195-273):PE + PSAP(195-273) ligands → PSAP(195-273):Ligands:PE
- PSAP(195-273) forms a dimer2 PSAP(195-273) → PSAP(195-273) dimer
- GM1 localizes to ILV membraneGM1-d18 → GM1
- GM1 localizes to endosome membraneGM1 → GM1-d18
- Beta-galactosidases hydrolyze GM2A:GA1 to GM2A:GA2H2O + GM2A:GA1 → Gal + GM2A:GA2
- bHEXA,bHEXB hydrolyze GM2A:GA2 to GM2A:LacCerH2O + GM2A:GA2 → GalNAc + GM2A:LacCer
- bHEXA,bHEXS hydrolyze GM2A:SM2GM2A:SM2 + H2O → GalNAc + SM3 + GM2A(32-193)
- ARSA removes sulfate from SM3SM3 + H2O → LacCer + SO4(2-)
- GLA hydrolyzes PSAP(195-273):Gal2Cer:PEH2O + PSAP(195-273):Gal2Cer:PE → Gal + GalCer + PSAP(195-273) dimer + PE 30,32,34
- PSAP(195-273) dimer binds PEPSAP(195-273) dimer + PE 30,32,34 → PSAP(195-273):PE
- ABCC1,ABCG2 transport C18-S1P to extracellular regionC18-S1P + ATP + H2O → C18-S1P + ADP + H+ + Pi
- GAL3ST1 transfers sulfate to GalCer,LacCerPAPS + GalCer,LacCer → PAP + H+ + SM3,SM4
- ST3GAL5 transfers NeuNAc to ceramidesLacCer/GalCer/GA1/GA2 + CMP-Neu5Ac → GM1/2/3/4 + CMP + H+
- B3GALT4 transfers Gal to gangliosidesGM2,GD2,GA2 + UDP-Gal → GM1,GD1b,GA1 + UDP + H+
- ST3GAL2,3 transfer Neu5Ac to gangliosidesCMP-Neu5Ac + GM1,GD1b,GA1 → CMP + GD1a,GM1b,GT1b + H+
- ST8SIA5 transfers Neu5Ac to gangliosidesCMP-Neu5Ac + GM1b,GD1a,GD3,GT1b → CMP + GQ1b,GT3,GD1c,GT1a + H+
- ST6GALNAC5,6 transfer Neu5Ac to GM1bGM1b + CMP-Neu5Ac → GD1alpha + CMP + H+
- FUT1,2 transfer fucose to gangliosidesGM1,GA1 + GDP-Fuc → Fuc-GM1, Fuc-GA1 + GDP + H+
- A4GALT transfers galactose to LacCerLacCer-d18 + UDP-Gal → Gb3Cer + UDP + H+
- B3GNT5 transfers GlcNAc to LacCerLacCer-d18 + UDP-GlcNAc → Lc3Cer + UDP + H+
- B4GALT5/6 transfer Gal to GlcCerUDP-Gal + GlcCer → UDP + H+ + LacCer-d18
- CERT1-2 transfers CERA from the ER membrane to the Golgi membraneCERA → CERA