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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.
glycine cleavage system deficiency
[Non-ketotic hyperglycinemia. Transient neonatal form]
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
Early myoclonic encephalopathy and nonketotic hyperglycinemia.
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
Natural history of nonketotic hyperglycinemia in 65 patients.
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: clinical and metabolic aspects.
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 Missense Mutations in 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 cases of glycine encephalopathy accompanied by pes equinovarus.
Metabolic Diseases
Two novel laboratory tests facilitating diagnosis of glycine encephalopathy (nonketotic hyperglycinemia).
Neoplasms
GCSH antisense regulation determines breast cancer cells' viability.
Neoplasms
Glycine decarboxylase regulates the maintenance and induction of pluripotency via metabolic control.
Neoplasms
In vivo MR studies of glycine and glutathione metabolism in a rat mammary tumor.
Neoplasms
SHMT2 drives glioma cell survival in ischaemia but imposes a dependence on glycine clearance.
Neural Tube Defects
Formate and its role in amino acid metabolism.
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.
Spasm
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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P20821 i.e. H-protein, lipoyl-carrier protein
UniProt
brenda
-
-
-
brenda
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
-
-
-
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
i.e. component H-protein
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
-
-
-
brenda
component H-protein
UniProt
brenda
i.e. dihydrolipoyl dehydrogenase component LpdA, cf. EC 1.8.1.4
UniProt
brenda
i.e. lipoyl-carrier protein component H-protein
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
-
-
-
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
-
-
-
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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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
-
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
-
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
-
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
physiological function
glycine cleavage system and cAMP receptor protein coregulate Cas3 of the CRISPR/Cas system and contribute to the defence against invasive genetic elements. Silencing of the glycine cleavage system encoded by the gcvTHP operon reduces Cas3 expression. Addition of N5,N10-methylene tetrahydrofolate activates Cas3 expression. A cAMP receptor protein encoded by Crp activates Cas3 expression via binding to the Cas3 promoter in response to cAMP concentration. The glycine cleavage system regulates Cas3 through association with cAMP receptor protein
physiological function
H-protein of the glycine cleavage system localizes into vesicles in the cell of Trimastix. When overexpressed in yeast, H- and P-protein are transported into mitochondrion. The first 16 amino acids of H-protein are necessary for this transport
physiological function
in an LpdA deletion mutant, inducible GCV enzyme activity is not detected. A D-3-phosphoglycerate dehydrogenase SerA/LpdA double mutant is unable to utilize glycine as a serine source and lacks detectable GCV enzyme activity
physiological function
in tobacco plants overexpressing the Arabidopsis thaliana H-protein, under controlled environment conditions an increase in biomass is evident. Targeted overexpression of the H-protein using the leaf-specific promoter ST-LS1 has a positive impact on biomass, but higher levels of overexpression of this protein driven by the constitutive CaMV 35S promoter result in a reduction in the growth of the plants. In the constitutive overexpressor plants, carbon allocation between soluble carbohydrates and starch is altered, as is the protein lipoylation of the pyruvate dehydrogenase and 2-oxoglutarate complexes
physiological function
mutations in Gldc result in severe or mild elevations of plasma glycine and model non-ketotic hyperglycinemia. Liver of Gldc-deficient mice accumulates glycine and numerous glycine derivatives, including multiple acylglycines. Levels of dysregulated metabolites increase with age and are normalised by liver-specific rescue of Gldc expression. Brain tissue exhibits increased abundance of glycine, as well as derivatives including guanidinoacetate. Elevation of brain tissue glycine occurs even in the presence of only mildly elevated plasma glycine in mice carrying a missense allele of Gldc. Treatment with benzoate enhances hepatic glycine conjugation thereby lowering plasma and tissue glycine. Administration of glycine conjugation pathway intermediate, cinnamate, similarly achieves normalisation of liver glycine derivatives and circulating glycine
physiological function
structure-based dynamic analysis of the induced release of the lipoate arm of protein H. Four major steps of the release process can be distinguished showing significantly different energy barriers and time scales. Mutations of key residue, Ser67 in protein H, leads to a bidirectional tuning of the release process
physiological function
T-protein knock out parasites do not show any growth defect in asexual, sexual and liver stages. T-protein is dispensable for parasite survival in vertebrate and invertebrate hosts
physiological function
the component T-protein catalyzes the degradation of the protein-bound intermediate (-CH2NH2 moiety of glycine) to a 1-carbon unit and NH3. The reaction is dependent on tetrahydrofolate. T-protein associates with H-protein forming a complex of one molecule each of T-protein and H-protein
physiological function
the glycine cleavage system CGS is highly activated to promote stem cell pluripotency and during somatic cell reprogramming. The expression of glycine dehydrogenase GldC, regulated by Sox2 and Lin28A, facilitates this activation. The activated GCS catabolizes glycine to fuel histone H3K4me3 modification, promoting the expression of pluripotency genes. The activated GCS helps to cleave excess glycine and prevents methylglyoxal accumulation, which stimulates senescence in stem cells and during reprogramming
physiological function
the isolated component P-protein can bind glycine and catalyze glycine decarboxylation but at extremely low rate. The product of glycine decarboxylation is methylamine. Methylamine can bind to P-protein, inhibiting the glycine decarboxylation. P-protein alone can also slightly catalyze the exchange of carboxyl carbon of glycine with CO2 and the exchange obeys a pingpong mechanism
physiological function
-
the lipoamide dehydrogenase component, cf. EC 1.8.1.4, is an indistinguishable constituent among alpha-keto acid dehydrogenase complexes and the glycine cleavage system in mitochondria in nature, and lipoamide dehydrogenase-mediated transfer of reducing equivalents might regulate alpha-keto acid oxidation as well as glycine oxidation
physiological function
the reversible glycine cleavage system in liver mitochondria involves four enzyme proteins designated as P-protein (a pyridoxal phosphate requiring protein), H-protein (a hydrogen carrier protein), L-protein (exhibiting a lipoamide dehydrogenase activity) and T-protein (a H4-folate requiring protein). All three protein fractions obtained during purification are essential for the overall reactions of glycine cleavage and glycine synthesis, while only P-, L-protein and H-protein are required for the glycine-14CO2 exchange
physiological function
-
the reversible glycine cleavage system is composed of four protein components named as P-, H-, L-, and T-protein, respectively. P-protein catalyzes the decarboxylation of glycine or its reverse reaction in the presence of H-protein, and T-protein participates in the formation of one carbon unit and ammonia or the reverse reaction
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Azize, N.A.; Ngah, W.Z.; Othman, Z.; Md Desa, N.; Chin, C.B.; Md Yunus, Z.; Mohan, A.; Hean, T.S.; Syed Zakaria, S.Z.; Lock-Hock, N.
Mutation analysis of glycine decarboxylase, aminomethyltransferase and glycine cleavage system protein-H genes in 13 unrelated families with glycine encephalopathy
J. Hum. Genet.
59
593-597
2014
Homo sapiens (P23378 and P23434 and P48728 and P09622), Homo sapiens
brenda
Varadarajan, N.M.; Sundaram, B.; Subramani, P.A.; Kalappa, D.M.; Ghosh, S.K.; Nagaraj, V.A.
Plasmodium berghei glycine cleavage system T-protein is non-essential for parasite survival in vertebrate and invertebrate hosts
Mol. Biochem. Parasitol.
197
50-55
2014
Plasmodium berghei (A0A122IGX3), Plasmodium berghei
brenda
Yoshida, T.; Kikuchi, G.
Significance of the glycine cleavage system in glycine and serine catabolism in avian liver
Arch. Biochem. Biophys.
145
658-668
1971
Columba sp., Gallus gallus (P15505)
brenda
Motokawa, Y.; Kikuchi, G.
Glycine metabolism by rat liver mitochondria. Reconstruction of the reversible glycine cleavage system with partially purified protein components
Arch. Biochem. Biophys.
164
624-633
1974
Rattus norvegicus
brenda
Kochi, H.; Seino, H.; Ono, K.
Inhibition of glycine oxidation by pyruvate, alpha-ketoglutarate, and branched-chain alpha-keto acids in rat liver mitochondria presence of interaction between the glycine cleavage system and alpha-keto acid dehydrogenase complexes
Arch. Biochem. Biophys.
249
263-272
1986
Rattus norvegicus
brenda
Fujiwara, K.; Okamura-Ikeda, K.; Motokawa, Y.
Amino acid sequence of the phosphopyridoxyl peptide from P-protein of the chicken liver glycine cleavage system
Biochem. Biophys. Res. Commun.
149
621-627
1987
Gallus gallus
brenda
Sato, K.; Yoshida, S.; Fujiwara, K.; Tada, K.; Tohyama, M.
Glycine cleavage system in astrocytes
Brain Res.
567
64-70
1991
Rattus norvegicus
brenda
Sakata, Y.; Owada, Y.; Sato, K.; Kojima, K.; Hisanaga, K.; Shinka, T.; Suzuki, Y.; Aoki, Y.; Satoh, J.; Kondo, H.; Matsubara, Y.; Kure, S.
Structure and expression of the glycine cleavage system in rat central nervous system
Brain Res. Mol. Brain Res.
94
119-130
2001
Rattus norvegicus
brenda
Zhang, H.; Li, Y.; Nie, J.; Ren, J.; Zeng, A.P.
Structure-based dynamic analysis of the glycine cleavage system suggests key residues for control of a key reaction step
Commun. Biol.
3
756
2020
Escherichia coli (P0A6T9)
brenda
Tan, Y.; Sou, N.; Tang, F.; Ko, H.; Yeh, W.; Peng, J.; Chiang, E.
Tracing metabolic fate of mitochondrial glycine cleavage system derived formate in vitro and in vivo
Int. J. Mol. Sci.
21
8808
2020
Homo sapiens (P23378 and P23434 and P48728 and P09622), Homo sapiens, Mus musculus (Q91W43 and O08749)
brenda
Steiert, P.S.; Stauffer, L.T.; Stauffer, G.V.
The lpd gene product functions as the L protein in the Escherichia coli glycine cleavage enzyme system
J. Bacteriol.
172
6142-6144
1990
Escherichia coli (P0A9P0)
brenda
Hiraga, K.; Kochi, H.; Motokawa, Y.; Kikuchi, G.
Enzyme complex nature of the reversible glycine cleavage system of cock liver mitochondria
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