1.4.1.27: glycine cleavage system
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For detailed information about glycine cleavage system, go to the full flat file.
Word Map on EC 1.4.1.27
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1.4.1.27
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hyperglycinemia
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nonketotic
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h-protein
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lipoic
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lipoylation
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one-carbon
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encephalopathy
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hydroxymethyltransferase
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lipoate
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dihydrolipoamide
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alpha-keto
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octanoylation
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2-oxoacids
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aminomethyltransferase
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5,10-methylenetetrahydrofolate
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dextromethorphan
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lipoyltransferase
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aminomethyl
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alpha-ketoacids
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1-carbon
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flaveria
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dimethylglycine
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medicine
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synthesis
- 1.4.1.27
- hyperglycinemia
-
nonketotic
- h-protein
-
lipoic
-
lipoylation
-
one-carbon
- encephalopathy
- hydroxymethyltransferase
- lipoate
- dihydrolipoamide
-
alpha-keto
-
octanoylation
- 2-oxoacids
- aminomethyltransferase
- 5,10-methylenetetrahydrofolate
- dextromethorphan
- lipoyltransferase
-
aminomethyl
- alpha-ketoacids
-
1-carbon
- flaveria
- dimethylglycine
- medicine
- synthesis
Reaction
Synonyms
Amt, At1g11860, At1g32470, At1g48030, AT2G35370, At4g33010, DLD, DLDH, GCSH, GCV, GcvH, GcvP, GCVT, GDCSH, GDCSP, GDH1, GDSCT, GLDC, glycine cleavage complex, H-protein, LpdA, P-protein, sll0171, slr1096, T-protein
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APPLICATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
medicine
synthesis
recombinant expression of H-protein in Escherichia coli. When the cells are cultured in medium supplemented with 30 microM lipoate, about 10% of the protein expressed is the correctly lipoylated active form, 10% is an inactive aberrantly modified form, and the remaining 80% is the unlipoylated apoform
medicine
in 14 patients from 13 families with clinical and biochemical features suggestive of glycine encephalopathy, mutations of the glycine cleavage system are identified. Seven patients (50%) have biallelic mutations in GldC gene, six patients (43%) have biallelic mutations in Amt gene and one patient (7%) has mutation identified in only one allele in GldC gene. Majority of the mutations in GldC and AMT are missense mutations and family-specific. No mutation is found in GcsH gene
medicine
in a patient with nonketotic hyperglycinemia, the H-protein purified from the liver is devoid of functional lipoic acid. H-protein from the patient is able to stimulate the P-protein-catalyzed exchange of the carboxyl carbon of glycine and CO2, although to a limited extent. P-Protein is inactivated when incubated with glycine in the presence of H-protein, and the inactivation is completely prevented when bicarbonate is further added. The inactivation is accompanied by a spectral change of P-protein. The inactivation of P-protein seems to reflect the formation of a ternary complex of P-protein, H-protein and aminomethyl moiety of glycine through a Schiff base linkage of the H-protein-bound aminomethyl moiety with the pyridoxal phosphate of P-protein
