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
2.3.1.-
1.1.1.100 3-oxoacyl-[acyl-carrier-protein] reductase (3R)-3-hydroxyacyl-[acyl-carrier protein]:NADP+ oxidoreductase
1.14.19.17 sphingolipid 4-desaturase dihydroceramide,ferrocytochrome b5:oxygen oxidoreductase (4,5-dehydrogenating)
1.6.5.10 NADPH dehydrogenase (quinone) NADPH:(quinone-acceptor) oxidoreductase
2.3.1.37 5-aminolevulinate synthase succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
2.3.1.50 serine C-palmitoyltransferase palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)
2.4.1.133 xylosylprotein 4-beta-galactosyltransferase UDP-galactose:O-beta-D-xylosylprotein 4-beta-D-galactosyltransferase
2.4.1.17 glucuronosyltransferase UDP-glucuronate beta-D-glucuronosyltransferase (acceptor-unspecific)
2.4.1.80 ceramide glucosyltransferase UDP-glucose:N-acylsphingosine D-glucosyltransferase
2.7.1.138 ceramide kinase ATP:ceramide 1-phosphotransferase
2.7.1.91 sphingosine kinase ATP:sphingoid base 1-phosphotransferase
3.1.4.39 alkylglycerophosphoethanolamine phosphodiesterase 1-alkyl-sn-glycero-3-phosphoethanolamine ethanolaminehydrolase
3.1.4.4 phospholipase D phosphatidylcholine phosphatidohydrolase
3.1.4.62 phosphatidylethanolamine phospholipase C phosphatidylethanolamine ethanolaminephosphohydrolase
3.1.6.2 steryl-sulfatase steryl-sulfate sulfohydrolase
3.2.1.18 exo-alpha-sialidase acetylneuraminyl hydrolase
3.2.1.46 galactosylceramidase D-galactosyl-N-acylsphingosine galactohydrolase
3.5.1.23 ceramidase N-acylsphingosine amidohydrolase
4.1.2.27 sphinganine-1-phosphate aldolase sphinganine-1-phosphate palmitaldehyde-lyase (phosphoethanolamine-forming)


Proteins

Protein ID name full name