Sucrase-Isomaltase Deficiency

Metabolites

ADP

Formula: C10H15N5O10P2 (427.02941500000003)

CAS ID: 58-64-0

alpha-D-Glucose

Formula: C6H12O6 (180.0633852)

CAS ID: 492-62-6

alpha-Maltose

Formula: C12H22O11 (342.11620619999997)

CAS ID: 4482-75-1

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

beta-D-Fructose

Formula: C6H12O6 (180.0633852)

CAS ID: 53188-23-1

D-Glucose 6-phosphate

Formula: C6H13O9P (260.0297178)

CAS ID: 56-73-5

D-Glucuronate

Formula: C6H10O7 (194.042651)

CAS ID: 6556-12-3

Glycerate 3-phosphate

Formula: C3H7O7P (185.9929402)

CAS ID: 820-11-1

Glycogen

Formula: C24H42O21 (666.2218482000001)

CAS ID: 9005-79-2

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

Sucrose

Formula: C12H22O11 (342.11620619999997)

CAS ID: 57-50-1

UDP

Formula: C9H14N2O12P2 (404.0021984)

CAS ID: 58-98-0

UDP-glucuronate

Formula: C15H22N2O18P2 (580.0342852)

CAS ID: 2616-64-0

UTP

Formula: C9H15N2O15P3 (483.968531)

CAS ID: 63-39-8

Isovalerylglucuronide

Formula: C11H18O8 (278.1001628)

CAS ID: 88070-93-3

Amylose

Formula: (C12H20O11)nC2H6 (3400695.5163906002)

CAS ID: 9005-82-7

Dextrin

Formula: C18H32O16 (504.1690272)

CAS ID: 9004-53-9

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
5.1.3.-
1.1.1.22 UDP-glucose 6-dehydrogenase UDP-alpha-D-glucose:NAD+ 6-oxidoreductase
2.4.1.1 glycogen phosphorylase (1->4)-alpha-D-glucan:phosphate alpha-D-glucosyltransferase
2.4.1.17 glucuronosyltransferase UDP-glucuronate beta-D-glucuronosyltransferase (acceptor-unspecific)
3.2.1.1 alpha-amylase 4-alpha-D-glucan glucanohydrolase
3.2.1.10 oligo-1,6-glucosidase oligosaccharide 6-alpha-glucohydrolase
3.2.1.31 beta-glucuronidase beta-D-glucuronoside glucuronosohydrolase
3.2.1.48 sucrose alpha-glucosidase sucrose-alpha-D-glucohydrolase
5.3.1.9 glucose-6-phosphate isomerase alpha-D-glucose-6-phosphate aldose-ketose-isomerase (configuration-inverting)
5.4.2.2 phosphoglucomutase (alpha-D-glucose-1,6-bisphosphate-dependent) alpha-D-glucose 1,6-phosphomutase


Proteins

Protein ID name full name