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Literature summary for 1.5.8.4 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)

Cloned (Comment) Organism
expression in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
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]

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Specific Activity [micromol/min/mg]

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

Substrates and Products (Substrate)

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

Synonyms Comment Organism
DMGDH
-
Homo sapiens

Turnover Number [1/s]

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