Glycosphingolipid metabolism

Sandhoff R, Sandhoff K. Emerging concepts of ganglioside metabolism. FEBS Lett. 2018 Dec;592(23):3835–64. doi: 10.1002/1873-3468.13114. PMID: 29802621.; Sandhoff R, Schulze H, Sandhoff K. Ganglioside Metabolism in Health and Disease. Prog Mol Biol Transl Sci. 2018;156():1–62. doi: 10.1016/bs.pmbts.2018.01.002. PMID: 29747811.; Gault CR, Obeid LM, Hannun YA. An overview of sphingolipid metabolism: from synthesis to breakdown. Adv Exp Med Biol. 2010;688():1–23. PMID: 20919643; PMCID: PMC3069696.

Metabolites

alpha-D-Galactose

Formula: C6H12O6 (180.0633852)

CAS ID: 3646-73-9

Calcium cation

Formula: Ca (39.962591)

CAS ID: 14127-61-8

Choline phosphate

Formula: C5H15NO4P (184.07386599999998)

CAS ID: 107-73-3

D-Glucose

Formula: C6H12O6 (180.0633852)

CAS ID: 50-99-7

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Magnesium cation

Formula: Mg (23.98505)

CAS ID: 22537-22-0

Manganese(2+)

Formula: Mn (54.938046)

CAS ID: 7439-96-5

N-Acetyl-D-galactosamine

Formula: C8H15NO6 (221.089933)

CAS ID: 14215-68-0

Sulfate

Formula: H2SO4 (97.9673812)

CAS ID: 7664-93-9

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

UDP-alpha-D-glucose(2-)

Formula: C15H22N2O17P2 (564.0393702)

CAS ID:

UDP-N-acetyl-D-galactosamine(2-)

Formula: C17H25N3O17P2 (605.0659180000001)

CAS ID:

NADH(2-)

Formula: C21H27N7O14P2 (663.1091182000001)

CAS ID:

NAD(1-)

Formula: C21H26N7O14P2 (662.1012936000001)

CAS ID: 53-84-9

UDP(3-)

Formula: C9H11N2O12P2 (400.9787246)

CAS ID: 86527-70-0

ADP(3-)

Formula: C10H12N5O10P2 (424.0059412)

CAS ID:

hydrosulfide

Formula: HS (32.979896600000004)

CAS ID: 15035-72-0



Enzyme

EC Number name full name note
2.4.1.-
2.4.1.206 lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase UDP-N-acetyl-D-glucosamine:beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide 3-beta-N-acetylglucosaminyltransferase
2.4.1.228 lactosylceramide 4-alpha-galactosyltransferase UDP-galactose:beta-D-galactosyl-(1->4)-D-glucosyl-(1<->1)-ceramide 4II-alpha-D-galactosyltransferase
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
2.4.1.62 ganglioside galactosyltransferase UDP-alpha-D-galactose:N-acetyl-beta-D-galactosaminyl-(1->4)-[alpha-N-acetylneuraminyl-(2->3)]-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide 3-beta-D-galactosyltransferase
2.4.1.79 globotriaosylceramide 3-beta-N-acetylgalactosaminyltransferase UDP-N-acetyl-D-galactosamine:alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide III3-beta-N-acetyl-D-galactosaminyltransferase
2.4.1.80 ceramide glucosyltransferase UDP-glucose:N-acylsphingosine D-glucosyltransferase
2.4.1.92 (N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase UDP-N-acetyl-D-galactosamine:1-O-[O-(N-acetyl-alpha-neuraminosyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide 4-beta-N-acetyl-D-galactosaminyltransferase
2.4.3.9 lactosylceramide alpha-2,3-sialyltransferase CMP-N-acetylneuraminate:beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide alpha-(2->3)-N-acetylneuraminyltransferase
3.1.4.12 sphingomyelin phosphodiesterase sphingomyelin cholinephosphohydrolase
3.2.1.18 exo-alpha-sialidase acetylneuraminyl hydrolase
3.2.1.45 glucosylceramidase D-glucosyl-N-acylsphingosine glucohydrolase
3.2.1.46 galactosylceramidase D-galactosyl-N-acylsphingosine galactohydrolase
3.5.1.23 ceramidase N-acylsphingosine amidohydrolase


Proteins

Protein ID name full name