Metachromatic Leukodystrophy (MLD)

Metabolites

3-Dehydrosphinganine

Formula: C18H37NO2 (299.2824142)

CAS ID: 16105-69-4

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

alpha-D-Galactose

Formula: C6H12O6 (180.0633852)

CAS ID: 3646-73-9

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

Choline phosphate

Formula: C5H15NO4P (184.07386599999998)

CAS ID: 107-73-3

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

D-Glucose

Formula: C6H12O6 (180.0633852)

CAS ID: 50-99-7

Ethanolamine phosphate

Formula: C2H8NO4P (141.0190938)

CAS ID: 1071-23-4

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hexadecanal

Formula: C16H32O (240.2453022)

CAS ID: 629-80-1

L-Serine

Formula: C3H7NO3 (105.0425912)

CAS ID: 56-45-1

NADP+

Formula: C21H29N7O17P3 (744.0832754)

CAS ID: 53-59-8

Sphinganine

Formula: C18H39NO2 (301.2980634)

CAS ID: 764-22-7

Sulfate

Formula: H2SO4 (97.9673812)

CAS ID: 7664-93-9

UDP

Formula: C9H14N2O12P2 (404.0021984)

CAS ID: 58-98-0

Cer(d18:1/18:0)

Formula: C36H71NO3 (565.5433656)

CAS ID: 2304-81-6

CerP(d18:1/18:0)

Formula: C36H72NO6P (645.5096982)

CAS ID: 128543-23-7

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
2.4.1.-
1.14.18.5 sphingolipid C4-monooxygenase dihydroceramide,ferrocytochrome b5:oxygen oxidoreductase (C4-hydroxylating)
1.14.19.17 sphingolipid 4-desaturase dihydroceramide,ferrocytochrome b5:oxygen oxidoreductase (4,5-dehydrogenating)
2.4.1.274 glucosylceramide beta-1,4-galactosyltransferase UDP-galactose:beta-D-glucosyl-(1<->1)-ceramide beta-1,4-galactosyltransferase
2.4.1.47 N-acylsphingosine galactosyltransferase UDP-galactose:N-acylsphingosine D-galactosyltransferase
2.7.8.27 sphingomyelin synthase ceramide:phosphatidylcholine cholinephosphotransferase
3.1.4.12 sphingomyelin phosphodiesterase sphingomyelin cholinephosphohydrolase
3.1.6.1 arylsulfatase (type I) aryl-sulfate sulfohydrolase
3.1.6.15 N-sulfoglucosamine-3-sulfatase N-sulfo-3-sulfoglucosamine 3-sulfohydrolase
3.2.1.49 alpha-N-acetylgalactosaminidase alpha-N-acetyl-D-galactosaminide N-acetylgalactosaminohydrolase
4.1.2.27 sphinganine-1-phosphate aldolase sphinganine-1-phosphate palmitaldehyde-lyase (phosphoethanolamine-forming)


Proteins

Protein ID name full name