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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
P
i
O-Phosphoethanolamine
Dihydroceramide
Ceramide (d18:1/18:0)
Sphingosine 1-phosphate
Sphingosine
ATP
ADP
ADP
ATP
P
i
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
H
2
O
Sulfate
D-Galactose
Galabiosylceramide
(d18:1/18:0)
N-Acetylneuraminyl-
galactosylceramide
(d18:1/18:0)
Sphinganine
CerP(d18:1/18:0)
H
2
O
P
i
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