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Information on EC 1.4.1.27 - glycine cleavage system
for references in articles please use BRENDA:EC1.4.1.27
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EC Tree 1 Oxidoreductases
1.4 Acting on the CH-NH2 group of donors
1.4.1 With NAD+ or NADP+ as acceptor
1.4.1.27 glycine cleavage system
IUBMB Comments
The glycine cleavage (GCV) system is a large multienzyme complex that belongs to the 2-oxoacid dehydrogenase complex family, which also includes EC 1.2.1.25, branched-chain α-keto acid dehydrogenase system, EC 1.2.1.105, 2-oxoglutarate dehydrogenase system, EC 1.2.1.104, pyruvate dehydrogenase system, and EC 2.3.1.190, acetoin dehydrogenase system. The GCV system catalyses the reversible oxidation of glycine, yielding carbon dioxide, ammonia, 5,10-methylenetetrahydrofolate and a reduced pyridine nucleotide. Tetrahydrofolate serves as a recipient for one-carbon units generated during glycine cleavage to form the methylene group. The GCV system consists of four protein components, the P protein (EC 1.4.4.2, glycine dehydrogenase (aminomethyl-transferring)), T protein (EC 2.1.2.10, aminomethyltransferase), L protein (EC 1.8.1.4, dihydrolipoyl dehydrogenase), and the non-enzyme H protein (lipoyl-carrier protein). The P protein catalyses the pyridoxal phosphate-dependent liberation of CO2 from glycine, leaving a methylamine moiety. The methylamine moiety is transferred to the lipoic acid group of the H protein, which is bound to the P protein prior to decarboxylation of glycine. The T protein catalyses the release of ammonia from the methylamine group and transfers the remaining C1 unit to tetrahydrofolate, forming 5,10-methylenetetrahydrofolate. The L protein then oxidizes the lipoic acid component of the H protein and transfers the electrons to NAD+, forming NADH.
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
- 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 Schemes
showSynonyms
glycine cleavage system, glycine cleavage complex, gdcsp, more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
At1g11860
gene name, aminomethyltransferase
At1g32470
gene name, H-protein isoform GCS-H1
At1g48030
gene name, dihydrolipoyl dehydrogenase
AT2G35370
At4g33010
gene name, glycine dehydrogenase
GCSH
GCV
GcvH
GCVT
GLDC
H-protein
AT2G35370
gene name, H-protein isoform GCS-H1
GCV
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
glycine + tetrahydrofolate + NAD+ = 5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
MetaCyc
folate transformations I, folate transformations II (plants), folate transformations III (E. coli), photorespiration I, photorespiration II, photorespiration III, reductive glycine pathway of autotrophic CO2 fixation
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SYSTEMATIC NAME
IUBMB Comments
glycine:NAD+ 2-oxidoreductase (tetrahydrofolate-methylene-adding)
The glycine cleavage (GCV) system is a large multienzyme complex that belongs to the 2-oxoacid dehydrogenase complex family, which also includes EC 1.2.1.25, branched-chain alpha-keto acid dehydrogenase system, EC 1.2.1.105, 2-oxoglutarate dehydrogenase system, EC 1.2.1.104, pyruvate dehydrogenase system, and EC 2.3.1.190, acetoin dehydrogenase system. The GCV system catalyses the reversible oxidation of glycine, yielding carbon dioxide, ammonia, 5,10-methylenetetrahydrofolate and a reduced pyridine nucleotide. Tetrahydrofolate serves as a recipient for one-carbon units generated during glycine cleavage to form the methylene group. The GCV system consists of four protein components, the P protein (EC 1.4.4.2, glycine dehydrogenase (aminomethyl-transferring)), T protein (EC 2.1.2.10, aminomethyltransferase), L protein (EC 1.8.1.4, dihydrolipoyl dehydrogenase), and the non-enzyme H protein (lipoyl-carrier protein). The P protein catalyses the pyridoxal phosphate-dependent liberation of CO2 from glycine, leaving a methylamine moiety. The methylamine moiety is transferred to the lipoic acid group of the H protein, which is bound to the P protein prior to decarboxylation of glycine. The T protein catalyses the release of ammonia from the methylamine group and transfers the remaining C1 unit to tetrahydrofolate, forming 5,10-methylenetetrahydrofolate. The L protein then oxidizes the lipoic acid component of the H protein and transfers the electrons to NAD+, forming NADH.
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
lipoic acid + NADH + H+
dihydrolipoic acid + NAD+
Substrates: assay of isolated L-protein, cf. EC 1.8.1.4
Products: -
Products: -
?
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
glycine + tetrahydrofolate + NAD+
Substrates: -
Products: -
Products: -
r
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
glycine + tetrahydrofolate + NAD+
-
Substrates: -
Products: -
Products: -
r
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
-
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
Substrates: -
Products: -
Products: -
r
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
-
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
-
Substrates: -
Products: -
Products: -
r
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
Substrates: -
Products: -
Products: -
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
glycine + tetrahydrofolate + NAD+
-
Substrates: -
Products: -
Products: -
r
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
-
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
Substrates: -
Products: -
Products: -
?
glycine + tetrahydrofolate + NAD+
5,10-methylenetetrahydrofolate + NH3 + CO2 + NADH
-
Substrates: -
Products: -
Products: -
r
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
pyridoxal 5'-phosphate
1 molecule per subunit of component P-protein
pyridoxal 5'-phosphate
-
P-protein component, cofactor is linked to residue Lys35, the pyridoxal 5'-phosphate-binding site has the His-Lys(PLP)-X structure
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Co2+
0.1 mM, 83% inhibition of the exchange of glycine carboxyl carbon with CO, catalyzed by glycine decarboxylase (P-protein) and aminomethyl carrier protein (H-protein)
Cu2+
0.1 mM, 100% inhibition of the exchange of glycine carboxyl carbon with CO, catalyzed by glycine decarboxylase (P-protein) and aminomethyl carrier protein (H-protein). Of the two partial reactions, decarboxylation of glycine yielding the H-protein-bound aminomethyl moiety is not significantly affected, but carboxylation of the H-protein-bound aminomethyl moiety to form glycine is strongly inhibited
Fe2+
0.1 mM, 20% inhibition of the exchange of glycine carboxyl carbon with CO, catalyzed by glycine decarboxylase (P-protein) and aminomethyl carrier protein (H-protein)
Ni2+
0.1 mM, 84% inhibition of the exchange of glycine carboxyl carbon with CO, catalyzed by glycine decarboxylase (P-protein) and aminomethyl carrier protein (H-protein)
Zn2+
0.1 mM, 99% inhibition of the exchange of glycine carboxyl carbon with CO, catalyzed by glycine decarboxylase (P-protein) and aminomethyl carrier protein (H-protein), competitive with both bicarbonate and H-protein and noncompetitive with glycine. Of the two partial reactions, decarboxylation of glycine yielding the H-protein-bound aminomethyl moiety is not significantly affected, but carboxylation of the H-protein-bound aminomethyl moiety to form glycine is strongly inhibited
additional information
additional information
no significant inhibition: Mn2+, Mg2+
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
2-oxo-3-methylvalerate
-
competitive with respect to glycine, presence leads to a decrease of apparent Km for NAD with a concomitant decrease of Vmax
2-oxoglutarate
-
competitive with respect to glycine, presence leads to a decrease of apparent Km for NAD with a concomitant decrease of Vmax
acetate
the presence of acetate and succinate in the growth medium results in a 50% reduction in inducible GCV enzyme activity
N-ethylmaleimide
the modification of the free lipoyl sulfhydryl group renders the intermediate complex inactive
succinate
the presence of acetate and succinate in the growth medium results in a 50% reduction in inducible GCV enzyme activity
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
lipoic acid
both glycine decarboxylation and the glycine-CO2 exchange catalyzed by P-protein are stimulated 100fold or more by the addition of lipoic acid
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis, Lactic
Clinical, biochemical, and genetic spectrum of seven patients with NFU1 deficiency.
Acidosis, Lactic
Novel mutations in IBA57 are associated with leukodystrophy and variable clinical phenotypes.
Brain Diseases
Use of Perampanel and a Ketogenic Diet in Nonketotic Hyperglycinemia: A Case Report.
Brain Diseases, Metabolic
Localized proton MR spectroscopic detection of nonketotic hyperglycinemia in an infant.
Carcinogenesis
Serine biosynthesis with one carbon catabolism and the glycine cleavage system represents a novel pathway for ATP generation.
Cystinosis
Cysteamine inhibition of [15N]-glycine turnover in cystinosis and of glycine cleavage system in vitro.
Dehydration
Gene expression analysis in Eucalyptus globulus exposed to drought stress in a controlled and a field environment indicates different strategies for short- and longer-term acclimation.
Epilepsies, Myoclonic
d-Glyceric aciduria does not cause nonketotic hyperglycinemia: A historic co-occurrence.
Genetic Diseases, Inborn
[Clinical and genetic analyses of a family with atypical nonketotic hyperglycinemia caused by compound heterozygous mutations in the GLDC gene].
glycine cleavage system deficiency
A novel mutation in the glycine decarboxylase gene in patient with non-ketotic hyperglycinemia.
glycine cleavage system deficiency
Progressive vacuolating glycine leukoencephalopathy with pulmonary hypertension.
Hydrocephalus
Impaired folate 1-carbon metabolism causes formate-preventable hydrocephalus in glycine decarboxylase-deficient mice.
Hyperglycinemia, Nonketotic
A novel intronic homozygous mutation in the AMT gene of a patient with nonketotic hyperglycinemia and hyperammonemia.
Hyperglycinemia, Nonketotic
A novel missense mutation in a neonate with nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
A novel mutation in the glycine decarboxylase gene in patient with non-ketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
A one-base deletion (183delC) and a missense mutation (D276H) in the T-protein gene from a Japanese family with nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
A single nucleotide substitution that abolishes the initiator methionine codon of the GLDC gene is prevalent among patients with glycine encephalopathy in Jerusalem.
Hyperglycinemia, Nonketotic
Atypical nonketotic hyperglycinemia confirmed by assay of the glycine cleavage system in lymphoblasts.
Hyperglycinemia, Nonketotic
Atypical nonketotic hyperglycinemia with normal cerebrospinal fluid to plasma glycine ratio.
Hyperglycinemia, Nonketotic
Chromosomal localization, structure, single-nucleotide polymorphisms, and expression of the human H-protein gene of the glycine cleavage system (GCSH), a candidate gene for nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Clinical heterogeneity of glycine encephalopathy in three Palestinian siblings: A novel mutation in the glycine decarboxylase (GLDC) gene.
Hyperglycinemia, Nonketotic
Crystal structure of human T-protein of glycine cleavage system at 2.0 A resolution and its implication for understanding non-ketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Defective glycine cleavage system in nonketotic hyperglycinemia. Occurrence of a less active glycine decarboxylase and an abnormal aminomethyl carrier protein.
Hyperglycinemia, Nonketotic
Delivery of a normal baby after preimplantation genetic diagnosis for non-ketotic hyperglycinaemia.
Hyperglycinemia, Nonketotic
Depletion of cerebral D-serine in non-ketotic hyperglycinemia: possible involvement of glycine cleavage system in control of endogenous D-serine.
Hyperglycinemia, Nonketotic
Determination of benzoic acid in serum or plasma by gas chromatography-mass spectrometry (GC/MS).
Hyperglycinemia, Nonketotic
Feasibility of prenatal diagnosis of nonketotic hyperglycinemia: existence of the glycine cleavage system in placenta.
Hyperglycinemia, Nonketotic
Genetic heterogeneity of the GLDC gene in 28 unrelated patients with glycine encephalopathy.
Hyperglycinemia, Nonketotic
Identification of a common mutation in Finnish patients with nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Identification of the first reported splice site mutation (IVS7-1G-->A) in the aminomethyltransferase (T-protein) gene (AMT) of the glycine cleavage complex in 3 unrelated families with nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Ketogenic diet in early myoclonic encephalopathy due to non ketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Localized proton MR spectroscopic detection of nonketotic hyperglycinemia in an infant.
Hyperglycinemia, Nonketotic
Magnetic resonance spectroscopy study of glycine pathways in nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Model mice for mild-form glycine encephalopathy: behavioral and biochemical characterizations and efficacy of antagonists for the glycine binding site of N-methyl D-aspartate receptor.
Hyperglycinemia, Nonketotic
Molecular genetic and potential biochemical characteristics of patients with T-protein deficiency as a cause of glycine encephalopathy (NKH).
Hyperglycinemia, Nonketotic
Mutation analysis of glycine decarboxylase, aminomethyltransferase and glycine cleavage system protein-H genes in 13 unrelated families with glycine encephalopathy.
Hyperglycinemia, Nonketotic
Mutation in SLC6A9 encoding a glycine transporter causes a novel form of non-ketotic hyperglycinemia in humans.
Hyperglycinemia, Nonketotic
Non-ketotic hyperglycinemia: an aim of the second generation of studies on pathogenesis.
Hyperglycinemia, Nonketotic
Nonketotic hyperglycinemia: A life-threatening disorder in Saudi newborns.
Hyperglycinemia, Nonketotic
Nonketotic hyperglycinemia: analyses of glycine cleavage system in typical and atypical cases.
Hyperglycinemia, Nonketotic
Nonketotic hyperglycinemia: two patients with primary defects of P-protein and T-protein, respectively, in the glycine cleavage system.
Hyperglycinemia, Nonketotic
Novel compound heterozygous LIAS mutations cause glycine encephalopathy.
Hyperglycinemia, Nonketotic
Novel mutations in the P-protein (glycine decarboxylase) gene in patients with glycine encephalopathy (non-ketotic hyperglycinemia).
Hyperglycinemia, Nonketotic
Nystagmus and subnormal electroretinographic response in nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Prenatal diagnosis of nonketotic hyperglycinemia: enzymatic analysis of the glycine cleavage system in chorionic villi.
Hyperglycinemia, Nonketotic
Rapid diagnosis of glycine encephalopathy by 13C-glycine breath test.
Hyperglycinemia, Nonketotic
Recurrent mutations in P- and T-proteins of the glycine cleavage complex and a novel T-protein mutation (N145I): a strategy for the molecular investigation of patients with nonketotic hyperglycinemia (NKH).
Hyperglycinemia, Nonketotic
Regulation of glycine metabolism by the glycine cleavage system and conjugation pathway in mouse models of non-ketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Structure of P-protein of the glycine cleavage system: implications for nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
Studies of the glycine cleavage enzyme system in brain from infants with glycine encephalopathy.
Hyperglycinemia, Nonketotic
The effect of hyperglycinemic treatment in captive-bred Vervet monkeys (Chlorocebus aethiops).
Hyperglycinemia, Nonketotic
Two cases of glycine encephalopathy accompanied by pes equinovarus.
Hyperglycinemia, Nonketotic
Two Novel GLDC Mutations in a Neonate with Nonketotic Hyperglycinemia.
Hyperglycinemia, Nonketotic
Two novel laboratory tests facilitating diagnosis of glycine encephalopathy (nonketotic hyperglycinemia).
Hyperglycinemia, Nonketotic
Two novel mutations in the glycine decarboxylase gene in a boy with classic nonketotic hyperglycinemia: case report.
Hyperglycinemia, Nonketotic
Use of Perampanel and a Ketogenic Diet in Nonketotic Hyperglycinemia: A Case Report.
Hyperglycinemia, Nonketotic
Valproate-induced chorea and encephalopathy in atypical nonketotic hyperglycinemia.
Hyperglycinemia, Nonketotic
[Clinical and genetic analyses of a family with atypical nonketotic hyperglycinemia caused by compound heterozygous mutations in the GLDC gene].
Infections
Vibrio cholerae ensures function of host proteins required for virulence through consumption of luminal methionine sulfoxide.
Intellectual Disability
Dextromethorphan in nonketotic hyperglycinaemia: metabolic variation confounds the dose-response relationship.
Malaria
Validation of a modified method for Bxb1 mycobacteriophage integrase-mediated recombination in Plasmodium falciparum by localization of the H-protein of the glycine cleavage complex to the mitochondrion.
Meningomyelocele
Genetic association of the glycine cleavage system genes and myelomeningocele.
Metabolic Diseases
Two novel laboratory tests facilitating diagnosis of glycine encephalopathy (nonketotic hyperglycinemia).
Neoplasms
Glycine decarboxylase regulates the maintenance and induction of pluripotency via metabolic control.
Neoplasms
SHMT2 drives glioma cell survival in ischaemia but imposes a dependence on glycine clearance.
Neural Tube Defects
Mutations in genes encoding the glycine cleavage system predispose to neural tube defects in mice and humans.
Neurologic Manifestations
Cysteamine inhibition of [15N]-glycine turnover in cystinosis and of glycine cleavage system in vitro.
Neurologic Manifestations
Model mice for mild-form glycine encephalopathy: behavioral and biochemical characterizations and efficacy of antagonists for the glycine binding site of N-methyl D-aspartate receptor.
Obesity
Obesity increases hepatic glycine dehydrogenase and aminomethyltransferase expression while dietary glycine supplementation reduces white adipose tissue in Zucker diabetic fatty rats.
Propionic Acidemia
Glycine cleavage system in ketotic hyperglycinemia: a reduction of H-protein activity.
Propionic Acidemia
The impaired expression of glycine decarboxylase in patients with hyperglycinemias.
Scrapie
2-Aminoacrylate Stress Induces a Context-Dependent Glycine Requirement in ridA Strains of Salmonella enterica.
Seizures
Dextromethorphan in nonketotic hyperglycinaemia: metabolic variation confounds the dose-response relationship.
Seizures
Use of Perampanel and a Ketogenic Diet in Nonketotic Hyperglycinemia: A Case Report.
Tuberculosis
X-ray structure determination of the glycine cleavage system protein H of Mycobacterium tuberculosis using an inverse Compton synchrotron X-ray source.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
additional information
-
Km value of the isolated P-protein for H-protein in 0.0074 mM, pH 6.2, 37Ā°C
-
40
glycine
value for the isolated P-protein, pH 6.6, 37Ā°C
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
14
assay of L-protein with lipoic acid, 25Ā°C, pH 6.3
additional information
specific maximum activities are 0.37 micromol per min and mg for the P-protein (bicarbonate-exchange reaction)
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
6.7
-
exchange reaction of the carboxyl carbon of glycine with CO2 catalyzed by the purified P-protein
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
Q5NHP0 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.210
UniProt
brenda
B0F467 i.e. component H-protein, B0F465 and B0F466 i.e. subunits 1 and 2 of glycine dehydrogenase component P-protein, cf. EC 1.4.4.2, B0F476 i.e. component L-protein, B0F460 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
P31023 i.e. dihydrolipoyl dehydrogenase component L-protein, cf. EC 1.8.1.4, P26969 i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2, P16048 i.e. component H-protein, P49364 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
P72740 i.e. dihydrolipoyl dehydrogenase component L-protein, cf. EC 1.8.1.4, P54261 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
Q9M5K3 i.e. dihydrolipoyl dehydrogenase component L-protein, cf. EC 1.8.1.4, Q94B78 i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2, P25855 i.e. component H-protein isoform H1, Q9LQL0 i.e. component H-protein isoform H3, O65396 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
i.e. dihydrolipoyl dehydrogenase component LpdA, cf. EC 1.8.1.4
UniProt
brenda
P27248 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10, P0A6T9 i.e. component H-protein, P33195 i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2
UniProt
brenda
i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
i.e. component P-protein, glycine dehydrogenase, cf. EC 1.4.4.2
UniProt
brenda
P15505 i.e. glycine dehydrogenase component P-protein, cf., EC 1.4.4.2, P11183 i.e. component H-protein, P28337 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10
UniProt
brenda
P23378 i.e. GldC, component P-protein, cf. EC 1.4.4.2, P09622 i.e. DldH, component L-protein, cf. 1.8.1.4, P48728 i.e. Amt, component T-protein, cf. EC 2.1.2.10
UniProt
brenda
P23378 i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2, P23434 i.e. component H-protein, P48728 i.e. aminomethyltransferase component T-protein, cf. EC 2.1.2.10, P09622 i.e. dihydrolipoyl dehydrogenase component L-protein, cf. EC 1.8.1.4
UniProt
brenda
i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2
UniProt
brenda
Q91W43 i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2, O08749 i.e. dihydrolipoyl dehydrogenase component L-protein, cf. EC 1.8.1.4
UniProt
brenda
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
in rat brain, the P-protein is confined to astrocytes. The intensity of astrocyte staining varies with regions, with the strongest staining in the hippocampus, the cerebellar cortex, the Bergmann glia in the cerebellum and the Muller cells in the retina
brenda
-
in rat brain, the P-protein is confined to astrocytes. The intensity of astrocyte staining varies with regions, with the strongest staining in the hippocampus, the cerebellar cortex, the Bergmann glia in the cerebellum and the Muller cells in the retina
brenda
-
P-protein mRNA is expressed mainly in glia-like cells, including Bergmann glias in the cerebellum, while T- and H-protein mRNAs are detected in both glial-like cells and neurons
brenda
-
in rat brain, the P-protein is confined to astrocytes. The intensity of astrocyte staining varies with regions, with the strongest staining in the hippocampus, the cerebellar cortex, the Bergmann glia in the cerebellum and the Muller cells in the retina
brenda
-
T- and H-protein mRNAs, but not P-protein mRNA, are expressed in the spinal cord
brenda
-
in rat brain, the P-protein is confined to astrocytes. The intensity of astrocyte staining varies with regions, with the strongest staining in the hippocampus, the cerebellar cortex, the Bergmann glia in the cerebellum and the Muller cells in the retina
brenda
-
molar ratios of P-, T-, and H-protein mRNA in cerebrocortical astrocyte culture are 5.7:1.0:2.4
brenda
-
in rat brain, the P-protein is confined to astrocytes. The intensity of astrocyte staining varies with regions, with the strongest staining in the hippocampus, the cerebellar cortex, the Bergmann glia in the cerebellum and the Muller cells in the retina
brenda
-
P-protein mRNA is expressed mainly in glia-like cells, including Bergmann glias in the cerebellum, while T- and H-protein mRNAs are detected in both glial-like cells and neurons
brenda
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in rat brain, the P-protein is confined to astrocytes. The intensity of astrocyte staining varies with regions, with the strongest staining in the hippocampus, the cerebellar cortex, the Bergmann glia in the cerebellum and the Muller cells in the retina
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
mitochondrion-like organelle
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Highest Expressing Human Cell Lines
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
metabolism
physiological function
metabolism
isotopic labeling to explore the in vitro and in vivo metabolic fate of the 2-carbon from [2-13C]glycine and [2-13C]serine. As the 2-carbon of glycine and serine is decarboxylated and catabolized via the GCS, the original 13C-labeled 2-carbon is transferred to tetrahydrofolate and yields methylene-tetrahydrofolate in the mitochondria. In hepatoma cell-lines, 2-carbon from glycine is incorporated into deoxythymidine, species of purines (deoxyadenine and deoxyguanine), and methionine
metabolism
isotopic labeling to explore the in vitro and in vivo metabolic fate of the 2-carbon from [2-13C]glycine and [2-13C]serine. In healthy mice, incorporation of GCS-derived formate from glycine 2-carbon is found in serine, methionine, dTMP, and methylcytosine in bone marrow DNA. Labeled glycine 2-carbon directly incorporates into serine, adenine and guanine (at C2 and C8 of purine) in the cytosol
metabolism
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liver mitochondria actively catalyze the cleavage of glycine into methylene-THF, CO2, and ammonia, but fail to appreciably catalyze CO2 formation from the alpha-carbon of glycine. The one-carbon compound derived from glycine in the avian livers is utilized largely for the synthesis of uric acid. The yields of 14C-hypoxanthine from 14C-glycine, especially from glycine-2-14C, are significantly increased by the addition of mitochondria to the soluble liver fraction, and under these conditions the ratio of the yields of 14C-hypoxanthine from glycine-l-14C and 2-14C rises to 1:2.3
metabolism
liver mitochondria actively catalyze the cleavage of glycine into methylene-THF, CO2, and ammonia, but fail to appreciably catalyze CO2 formation from the alpha-carbon of glycine. The one-carbon compound derived from glycine in the avian livers is utilized largely for the synthesis of uric acid. The yields of 14C-hypoxanthine from 14C-glycine, especially from glycine-2-14C, are significantly increased by the addition of mitochondria to the soluble liver fraction, and under these conditions the ratio of the yields of 14C-hypoxanthine from glycine-l-14C and 2-14C rises to 1:2.3
metabolism
the amino group of glycine is retained in the intermediate and released as ammonia in the second partial reaction catalyzed by T-protein. The formation of ammonia accompanies the stoichiometric formation of 5,10-methylenetetrahydrofolate from the methylene carbon of glycine and tetrahydrofolate. The reaction proceeds through a sequential mechanism. Km values for the intermediate complex and tetrahydrofolate are 2.2 and 50 microM, respectively. In the absence of tetrahydrofolate, T-protein catalyzes the stoichiometric formation of ammonia and formaldehyde from the intermediate although the velocity is extremely low. The addition of tetrahydrofolate increases the rate about 2400fold
metabolism
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the enzyme is one of the four components that form the glycine cleavage complex (GCS), essential for the synthesis of C1 (one-carbon units) for cell metabolism, by the oxidative cleavage of glycine. The glycine cleavage complex (GCS), in cooperation with GCA (serine hydroxymethyltransferase) regulates the endogenous levels of glycine and C1 units in the cell. This system comprises four loosely associated proteins, namely GcvP (a pyridoxal phosphate-containing protein), GcvH (a protein that carries aminomethyl intermediate), GcvT (protein required for tetrahydrofolate-dependent reaction) and GcvL (a lipoamide dehydrogenase). GcvP decarboxylates glycine and relocates the remaining methylamine moiety to the lipoyl group of GcvH
physiological function
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construction of a genome-scale model of human cell metabolism to investigate the potential metabolic alterations in cells using net zero ATP glycolysis. A pathway for ATP generation involves reactions from serine biosynthesis, one-carbon metabolism and the glycine cleavage system, and is transcriptionally upregulated in an inducible murine model of Myc-driven liver tumorigenesis. This pathway has a predicted two-fold higher flux rate in cells using net zero ATP glycolysis than those using standard glycolysis and generates twice as much ATP with significantly lower rate of lactate, but higher rate of alanine secretion
physiological function
deletion of the gcvT homolog, i.e. T-protein, in attenuated and virulent Francisella tularensis strains. Deletion mutants are auxotrophic for serine but behave similar to wild-type strains with respect to host cell invasion, intracellular replication, and stimulation of TNFalpha. The glycine cleavage system is required for the pathogenesis of virulent Francisella tularensis in a murine model. Deletion of the gcvT gene delays mortality and lowers bacterial burden, particularly in the liver and bloodstream