Salla Disease/Infantile Sialic Acid Storage Disease

Metabolites

Acetate

Formula: C2H4O2 (60.0211284)

CAS ID: 64-19-7

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

Ammonia

Formula: NH3 (17.0265478)

CAS ID: 7664-41-7

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

beta-D-Fructose

Formula: C6H12O6 (180.0633852)

CAS ID: 53188-23-1

CMP-N-acetylneuraminate

Formula: C20H31N4O16P (614.1472616)

CAS ID: 3063-71-6

CTP

Formula: C9H16N3O14P3 (482.9845146)

CAS ID: 65-47-4

D-Fructose 6-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 643-13-0

D-Glucosamine

Formula: C6H13NO5 (179.0793688)

CAS ID: 3416-24-8

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

L-Glutamate

Formula: C5H9NO4 (147.0531554)

CAS ID: 56-86-0

L-Glutamine

Formula: C5H10N2O3 (146.069139)

CAS ID: 56-85-9

N-Acetyl-D-glucosamine

Formula: C8H15NO6 (221.089933)

CAS ID: 7512-17-6

N-Acetyl-D-mannosamine

Formula: C8H15NO6 (221.089933)

CAS ID: 3615-17-6

Phosphoenolpyruvate

Formula: C3H5O6P (167.98237600000002)

CAS ID: 138-08-9

Pyruvate

Formula: C3H4O3 (88.0160434)

CAS ID: 127-17-3

UDP

Formula: C9H14N2O12P2 (404.0021984)

CAS ID: 58-98-0

UTP

Formula: C9H15N2O15P3 (483.968531)

CAS ID: 63-39-8

Chitobiose

Formula: C15H26N2O12 (426.1485676)

CAS ID: 577-76-4

diphosphate

Formula: O7P2 (173.911931)

CAS ID: 14000-31-8

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
2.7.1.-
2.6.1.16 glutamine-fructose-6-phosphate transaminase (isomerizing) L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
2.7.1.1 hexokinase ATP:D-hexose 6-phosphotransferase
2.7.1.59 N-acetylglucosamine kinase ATP:N-acetyl-D-glucosamine 6-phosphotransferase
2.7.1.60 N-acylmannosamine kinase ATP:N-acyl-D-mannosamine 6-phosphotransferase
2.7.7.43 N-acylneuraminate cytidylyltransferase CTP:N-acylneuraminate cytidylyltransferase
3.1.3.29 N-acylneuraminate-9-phosphatase N-acylneuraminate-9-phosphate phosphohydrolase
3.2.1.14 chitinase (1->4)-2-acetamido-2-deoxy-beta-D-glucan glycanohydrolase
3.2.1.183 UDP-N-acetylglucosamine 2-epimerase (hydrolysing) UDP-N-acetyl-alpha-D-glucosamine hydrolase (2-epimerising)
3.2.1.52 beta-N-acetylhexosaminidase beta-N-acetyl-D-hexosaminide N-acetylhexosaminohydrolase
3.5.1.25 N-acetylglucosamine-6-phosphate deacetylase N-acetyl-D-glucosamine-6-phosphate amidohydrolase
4.1.3.3 N-acetylneuraminate lyase aceneuramate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
5.1.3.8 N-acylglucosamine 2-epimerase N-Acyl-D-glucosamine 2-epimerase


Proteins

Protein ID name full name