Leloir pathway

Segal S. Of mice and men: Galactosemia. Molecular Genetics and Metabolism. 2006 Dec;89(4):401–2. doi: 10.1016/j.ymgme.2006.06.005.; Ai Y, Zheng Z, O'Brien-Jenkins A, Bernard DJ, Wynshaw-Boris T, Ning C, Reynolds R, Segal S, Huang K, Stambolian D. A mouse model of galactose-induced cataracts. Hum Mol Genet. 2000 Jul 22;9(12):1821–7. doi: 10.1093/hmg/9.12.1821. PMID: 10915771.; Leslie ND, Yager KL, McNamara PD, Segal S. A Mouse Model of Galactose-1-Phosphate Uridyl Transferase Deficiency. Biochemical and Molecular Medicine. 1996 Oct;59(1):7–12. doi: 10.1006/bmme.1996.0057.; Leloir LF. Far away and long ago. Annu Rev Biochem. 1983;52():1–15. doi: 10.1146/annurev.bi.52.070183.000245. PMID: 6351722.

Metabolites

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

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

UDP-alpha-D-glucose(2-)

Formula: C15H22N2O17P2 (564.0393702)

CAS ID:



Enzyme

EC Number name full name note
2.7.1.157 N-acetylgalactosamine kinase ATP:N-acetyl-D-galactosamine 1-phosphotransferase
2.7.1.6 galactokinase ATP:D-galactose 1-phosphotransferase
2.7.7.12 UDP-glucose-hexose-1-phosphate uridylyltransferase UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
5.1.3.2 UDP-glucose 4-epimerase UDP-glucose 4-epimerase
5.1.3.7 UDP-N-acetylglucosamine 4-epimerase UDP-N-acetyl-D-glucosamine 4-epimerase


Proteins

Protein ID name full name