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Literature summary for 5.4.2.8 extracted from

  • Silvaggi, N.R.; Zhang, C.; Lu, Z.; Dai, J.; Dunaway-Mariano, D.; Allen, K.N.
    The X-ray crystal structures of human alpha-phosphomannomutase 1 reveal the structural basis of congenital disorder of glycosylation type 1a (2006), J. Biol. Chem., 281, 14918-14926.
    View publication on PubMed

Application

Application Comment Organism
medicine analysis of clinically important mutations involved in congential disorder of glycosylation type 1a Homo sapiens

Crystallization (Commentary)

Crystallization (Comment) Organism
isoform alpha-PMM1 in the open conformation, with and without bound substrate alpha-D-mannose 1-phosphate. Protein consists of two domains, the cap and the core. Substrate phosphate group is at a positively charged site of the cap domain, suggesting that substrate binds first to the cap and then is swept into the active site, thereby facilitating its closure over the core domain Homo sapiens

Protein Variants

Protein Variants Comment Organism
D188G | mutant of isoform PMM2, 2% of wild-type activiy, involved in congential disorder of glycosylation type 1a Homo sapiens
D65Y mutant of isoform PMM2, involved in congential disorder of glycosylation type 1a Homo sapiens
F119L mutant of isoform PMM2, involved in congential disorder of glycosylation type 1a Homo sapiens
R141H mutant of isoform PMM2, 0.4% of wild-type activiy, involved in congential disorder of glycosylation type 1a Homo sapiens
R162W mutant of isoform PMM2, involved in congential disorder of glycosylation type 1a Homo sapiens
V129M mutant of isoform PMM2, involved in congential disorder of glycosylation type 1a Homo sapiens
V231M mutant of isoform PMM2, loss of stability at 40°C, involved in congential disorder of glycosylation type 1a Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.0075
-
D-glucose 1-phosphate isoform alpha-PMM1, pH 6.5, 25°C Homo sapiens
0.0135
-
D-glucose-1-phosphate isoform alpha-PMM2, pH 6.5, 25°C Homo sapiens
0.016
-
D-mannose-1-phosphate isoform alpha-PMM2, pH 6.5, 25°C Homo sapiens
0.054
-
D-mannose 1-phosphate isoform alpha-PMM1, pH 6.5, 25°C Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens O15305 isoform alpha-PMM2
-
Homo sapiens Q92871 isoform alpha-PMM1
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
D-glucose 1-phosphate
-
Homo sapiens D-glucose 6-phosphate
-
?
D-glucose-1-phosphate
-
Homo sapiens D-glucose-6-phosphate
-
?
D-Mannose 1-phosphate
-
Homo sapiens D-Mannose 6-phosphate
-
?

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
1.72
-
D-glucose-1-phosphate isoform alpha-PMM2, pH 6.5, 25°C Homo sapiens
2.85
-
D-glucose 1-phosphate isoform alpha-PMM1, pH 6.5, 25°C Homo sapiens
3.9
-
D-mannose-1-phosphate isoform alpha-PMM2, pH 6.5, 25°C Homo sapiens
4.4
-
D-mannose 1-phosphate isoform alpha-PMM1, pH 6.5, 25°C Homo sapiens