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Literature summary extracted from

  • McAndrew, R.P.; Vockley, J.; Kim, J.J.
    Molecular basis of dimethylglycine dehydrogenase deficiency associated with pathogenic variant H109R (2008), J. Inherit. Metab. Dis., 31, 761-768.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.5.8.4 expression in Escherichia coli Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.5.8.4 H109R natural polymorphism identified in a dimethylglycine dehydrogenase-deficient patient. Mutant shows only 47% of the wild-type level of bound flavin, a 27fold decrease in specific activity and a 65fold increase in Km value Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.5.8.4 0.039
-
 N,N-dimethylglycine and second value 15.4 mM, wild-type, pH 7.5, 25°C Homo sapiens
1.5.8.4 0.045
-
 N,N-dimethylglycine and second value 1.10 mM, wild-type, presence of tetrahydrofolate, pH 7.5, 25°C Homo sapiens
1.5.8.4 1.1
-
 N,N-dimethylglycine and first value 0.045 mM, wild-type, presence of tetrahydrofolate, pH 7.5, 25°C Homo sapiens
1.5.8.4 2.5 5 N,N-dimethylglycine mutant H109R, pH 7.5, 25°C Homo sapiens
1.5.8.4 15.4
-
 N,N-dimethylglycine and first value 0.039 mM, wild-type, pH 7.5, 25°C Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
1.5.8.4 Homo sapiens
-
-
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
1.5.8.4 0.0068
-
 mutant H109R, pH 7.5, 25°C Homo sapiens
1.5.8.4 0.165
-
 wild-type, pH 7.5, 25°C Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.5.8.4 N,N-dimethylglycine + ferricenium hexafluorophosphate + H2O
-
 Homo sapiens sarcosine + formaldehyde + reduced ferricenium hexafluorophosphate
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.5.8.4 DMGDH
-
 Homo sapiens

Turnover Number [1/s]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
1.5.8.4 0.01
-
 N,N-dimethylglycine mutant H109R, pH 7.5, 25°C Homo sapiens
1.5.8.4 0.225
-
 N,N-dimethylglycine wild-type, presence of tetrahydrofolate, pH 7.5, 25°C Homo sapiens
1.5.8.4 0.3
-
 N,N-dimethylglycine wild-type, pH 7.5, 25°C Homo sapiens