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Serine palmitoyltransferase 2 Serine palmitoyltransferase 1 3- ketodihydrosphingosine reductase Sphingosine kinase 2 Sphingosine- 1-phosphate phosphatase 2 Lipid phosphate phosphohydrolase 1 Sphingosine- 1-phosphate lyase 1 Sphingolipid delta(4)- desaturase/C4- hydroxylase DES2 Sphingosine- 1-phosphate lyase 1 Sphingosine kinase 2 Sphingosine- 1-phosphate phosphatase 2 Lipid phosphate phosphohydrolase 1 Alkaline ceramidase 1 Phosphatidylcholine:ceramide cholinephosphotransferase 1 Ectonucleotide pyrophosphatase/phosphodiesterase family member 7 2- hydroxyacylsphingosine 1-beta- galactosyltransferase Galactocerebrosidase Ceramide glucosyltransferase Beta- galactosidase Beta-1,4- galactosyltransferase 6 Galactosylceramide sulfotransferase Arylsulfatase A Alpha- galactosidase A Sialidase-3 Alkaline ceramidase 3 Lipid phosphate phosphohydrolase 1 Sphingosine- 1-phosphate phosphatase 2 Ceramide kinase Galactosylceramide sulfotransferase Glucosylceramidase L-Serine Palmitoyl-CoA 3-Dehydrosphinganine Sphinganine NADPH NADP ATP ADP Sphinganine 1-phosphate ADP ATP Pi O-Phosphoethanolamine Dihydroceramide Ceramide (d18:1/18:0) Sphingosine 1-phosphate Sphingosine ATP ADP ADP ATP Pi SM(d18:1/18:0) Galactosylceramide (d18:1/18:0) Lactosylceramide (d18:1/18:0) Phosphoadenosine phosphosulfate 3-O-Sulfogalactosylceramide (d18:1/18:0) Adenosine 3',5'-diphosphate H2O Sulfate D-Galactose Galabiosylceramide (d18:1/18:0) N-Acetylneuraminyl- galactosylceramide (d18:1/18:0) Sphinganine CerP(d18:1/18:0) H2O Pi ATP ADP Phosphoadenosine phosphosulfate Adenosine 3',5'-diphosphate Galabiosylceramide sulfate (d18:1/18:0) Pyridoxal 5'-phosphate Magnesium Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Magnesium Magnesium Calcium Magnesium Glucosylceramide (d18:1/18:0) Glycine and Serine Metabolism Zinc Calcium Endoplasmic Reticulum Golgi complex Golgi complex Lysosome Lysosome Symptoms include anemia, fatigue, hepatomegaly, splenomegaly, and brain damage Liver Brain Red Blood Cells
Endoplasmic Reticulum SPTLC2 SPTLC1 KDSR SPHK2 SGPP2 PPAP2A SGPL1 DEGS2 SGPL1 SPHK2 SGPP2 PPAP2A ACER1 SGMS1 ENPP7 UGT8 GALC UGCG GLB1 B4GALT6 GAL3ST1 ARSA GLA NEU3 ACER3 PPAP2A SGPP2 CERK GAL3ST1 GBA L-Serine Palmitoyl-CoA 3- Dehydrosphinganine Sphinganine NADPH NADP Adenosine triphosphate Adenosine diphosphate Sphinganine 1-phosphate Adenosine diphosphate Adenosine triphosphate Phosphate O- Phosphoethanolamine Dihydroceramide Ceramide (d18:1/18:0) Sphingosine 1-phosphate Sphingosine Adenosine triphosphate Adenosine diphosphate Adenosine diphosphate Adenosine triphosphate Phosphate SM(d18:1/18:0) Galactosylceramide (d18:1/18:0) Lactosylceramide (d18:1/18:0) Phosphoadenosine phosphosulfate 3-O- Sulfogalactosylceramide (d18:1/18:0) Adenosine 3',5'- diphosphate Water Sulfate D-Galactose Galabiosylceramide (d18:1/18:0) N- Acetylneuraminyl- galactosylceramide (d18:1/18:0) Sphinganine CerP(d18:1/18:0) Water Phosphate Adenosine triphosphate Adenosine diphosphate Phosphoadenosine phosphosulfate Adenosine 3',5'- diphosphate Galabiosylceramide sulfate (d18:1/18:0) Glucosylceramide (d18:1/18:0) Glycine and Serine Metabolism
SPTLC2 SPTLC1 KDSR SPHK2 SGPP2 PPAP2A SGPL1 DEGS2 SGPL1 SPHK2 SGPP2 PPAP2A ACER1 SGMS1 ENPP7 UGT8 GALC UGCG GLB1 B4GALT6 GAL3ST1 ARSA GLA NEU3 ACER3 PPAP2A SGPP2 CERK GAL3ST1 GBA Ser COA16:0 3-DHPG d18:0 NADPH NADP ATP ADP Sphg-1P ADP ATP Pi Ethamp DHCeram Ceram18 S1P Sphingo ATP ADP ADP ATP Pi Sphingo Galacto Lacto18 Paps 3OSs A3'5'PP H2O SO4 D-Gal Galabio NAG d18:0 CerP18 H2O Pi ATP ADP Paps A3'5'PP GalacSO Pyr-5'P Mg2+ Pyr-5'P Pyr-5'P Mg2+ Mg2+ Ca2+ Mg2+ Glucosy PW000157 Zinc Ca2+ Endoplasmic Reticulum Golgi complex Golgi complex Lysosome Lysosome Symptoms include anemia, fatigue, hepatomegaly, splenomegaly, and brain damage Liver Brain Red Blood Cells
Endoplasmic Reticulum SPTLC2 SPTLC1 KDSR SPHK2 SGPP2 PPAP2A SGPL1 DEGS2 SGPL1 SPHK2 SGPP2 PPAP2A ACER1 SGMS1 ENPP7 UGT8 GALC UGCG GLB1 B4GALT6 GAL3ST1 ARSA GLA NEU3 ACER3 PPAP2A SGPP2 CERK GAL3ST1 GBA Ser COA16:0 3-DHPG d18:0 NADPH NADP ATP ADP Sphg-1P ADP ATP Pi Ethamp DHCeram Ceram18 S1P Sphingo ATP ADP ADP ATP Pi Sphingo Galacto Lacto18 Paps 3OSs A3'5'PP H2O SO4 D-Gal Galabio NAG d18:0 CerP18 H2O Pi ATP ADP Paps A3'5'PP GalacSO Glucosy PW000157