LPP1
PPAP2A
UniProt:O14494 PPAP2A
DEGS1
DES1
MIG15
MLD
UniProt:O15121 DEGS1
KIAA1252
SGPL1
UniProt:O95470 SGPL1
ASM
SMPD1
UniProt:P17405 SMPD1
GM2A
UniProt:P17900 GM2A
GALC
UniProt:P54803 GALC
CK1G2
CSNK1G2
UniProt:P78368 CSNK1G2
FVT1
KDSR
UniProt:Q06136 KDSR
PKD
PKD1
PRKCM
PRKD1
UniProt:Q15139 PRKD1
UGCG
UniProt:Q16739 UGCG
ACER2
ASAH3L
UniProt:Q5QJU3 ACER2
C14orf66
DEGS2
UniProt:Q6QHC5 DEGS2
ENPP7
UniProt:Q6UWV6 ENPP7
MOB
SGMS1
SMS1
TMEM23
UniProt:Q86VZ5 SGMS1
FGE
PSEC0152
SUMF1
UniProt:Q8NBK3 SUMF1
SGMS2
SMS2
UniProt:Q8NHU3 SGMS2
CERK
KIAA1646
UniProt:Q8TCT0 CERK
ACER1
ASAH3
UniProt:Q8TDN7 ACER1
CST
GAL3ST1
UniProt:Q99999 GAL3ST1
CBG
CBGL1
GBA3
UniProt:Q9H227 GBA3
AD035
GBA2
KIAA1605
UniProt:Q9HCG7 GBA2
ASAH2
HNAC1
UniProt:Q9NR71 ASAH2
ACER3
APHC
PHCA
UniProt:Q9NUN7 ACER3
NEU3
UniProt:Q9UQ49 NEU3
NEU2
UniProt:Q9Y3R4 NEU2
SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
3-ketosphinganine + NADPH + H+ => sphinganine + NADP+
CERT + ATP => monophospho-CERT + ADP
CERT [ER] + ceramide [ER] => CERT:ceramide [ER]
âCERTâ protein binds ceramide associated with the endoplasmic reticulum membrane
CERT:PPM1L:VAPA/B [ER] => CERT [cytosol] + PPM1L:VAPA/B [ER]
âCERTâ dissociates from the endoplasmic reticulum
CERT:ceramide [ER] => ceramide [Golgi] + CERT [ER]
âCERTâ protein releases its bound ceramide into the membrane of the Golgi apparatus
ceramide + H2O <=> stearate + sphingosine [endoplasmic reticulum]
ceramide + H2O => stearate + sphingosine [Golgi]
dihydroceramide + NAD(P)H + H+ + O2 => ceramide + NAD(P)+ + H2O
dihydroceramide + NADPH + H+ + O2 => phytoceramide + NADP+ + H2O
monophospho-CERT + 2 ATP => multiphospho-CERT + 2 ADP
multiphospho-CERT + PPM1L:VAPA/B => multiphospho-CERT:PPM1L:VAPA/B
multiphospho-CERT:PPM1L:VAPA/B + 3 H2O => CERT:PPM1L:VAPA/B + 3 orthophosphate
palmitoyl-CoA + serine => 3-ketosphinganine + CoASH + CO2
phosphatidylcholine + ceramide <=> sphingomyelin + diacylglycerol [SGMS1]
phosphatidylcholine + ceramide <=> sphingomyelin + diacylglycerol [SGMS2]
phytoceramide + H2O => stearate + phytosphingosine
sphinganine (dihydrosphingosine) +ATP => sphinganine 1-phosphate + ADP
sphinganine + stearyl-CoA => dihydroceramide + CoASH
sphinganine 1-phosphate + H2O => sphinganine + orthophosphate
sphinganine 1-phosphate => phosphoethanolamine + hexadecanal
sphingosine +ATP => sphingosine 1-phosphate + ADP
sphingosine 1-phosphate + H2O => sphingosine + orthophosphate [cytosolic - PPAP]
sphingosine 1-phosphate + H2O => sphingosine + orthophosphate [cytosolic - SGPP]
sphingosine 1-phosphate + H2O => sphingosine + orthophosphate [extracellular]
sphingosine 1-phosphate => phosphoethanolamine + hexadec-2-enal
Beta-galactosidase hydrolyses GM1 to GM2
Ganglioside GM2 activator presents GM2 to hexosaminidase for cleavage
Hexosaminidase A cleaves GalNAc from GM2 to form GM3
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides
Both hexosaminidase A and B can cleave GalNAc from globoside
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers
Active ARSA translocates to the lysosome
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome)
Sphingomyelin phosphodiesterase 2 and 3 (SMPD2 and 3) hydrolyse sphingomyelin to ceramide (plasma membrane)
Sphingomyelin phosphodiesterase 4 (SMPD4) hydrolyses sphingomyelin to ceramide (ER membrane)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
Glucosylceramidase 2 cleaves the glucosidic bond of glucocerebroside to form ceramide (plasma membrane)
Glucosylceramidase 3 cleaves the glucosidic bond of glucocerebroside to form ceramide (cytosol)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
Ectonucleotide pyrophosphatase/phosphodiesterase 7 (ENPP7) hydrolyses sphingomyelin
Neu5Ac is cleaved from GM3 by NEU2 to form a globoside (cytosol)
Neu5Ac is cleaved from GM3 by NEU3 to form globoside (plasma membrane)
Neutral ceramidase hydrolyses ceramide into sphingosine and free fatty acid (plasma membrane)
Steryl sulfatase hydrolyses sulfate from steroid sulfates
Ceramide glucosyltransferase (UGCG) catalyses the transfer of glucose to ceramide
Ceramide kinase (CERK) mediates the phosphorylation of ceramide
Galactosylceramide sulfotransferase (GAL3ST1) mediates the sulfation of membrane glycolipids
Sphingolipid metabolism
Sphingolipid de novo biosynthesis
Glycosphingolipid metabolism