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Glycine cleavage system information


Glycine cleavage H-protein
refined structures at 2 angstroms and 2.2 angstroms of the two forms of the h-protein, a lipoamide-containing protein of the glycine decarboxylase
Identifiers
SymbolGCV_H
PfamPF01597
Pfam clanCL0105
InterProIPR002930
SCOP21htp / SCOPe / SUPFAM
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary
Glycine cleavage T-protein, Aminomethyltransferase folate-binding domain
crystal structure of a component of glycine cleavage system: t-protein from pyrococcus horikoshii ot3 at 1.5 a resolution
Identifiers
SymbolGCV_T
PfamPF01571
Pfam clanCL0289
InterProIPR006222
SCOP21pj5 / SCOPe / SUPFAM
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary
Glycine cleavage T-protein C-terminal barrel domain
crystal structure of t-protein of the glycine cleavage system
Identifiers
SymbolGCV_T_C
PfamPF08669
InterProIPR013977
SCOP21pj5 / SCOPe / SUPFAM
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary

The glycine cleavage system (GCS) is also known as the glycine decarboxylase complex or GDC. The system is a series of enzymes that are triggered in response to high concentrations of the amino acid glycine.[1] The same set of enzymes is sometimes referred to as glycine synthase when it runs in the reverse direction to form glycine.[2] The glycine cleavage system is composed of four proteins: the T-protein, P-protein, L-protein, and H-protein. They do not form a stable complex,[3] so it is more appropriate to call it a "system" instead of a "complex". The H-protein is responsible for interacting with the three other proteins and acts as a shuttle for some of the intermediate products in glycine decarboxylation.[2] In both animals and plants, the glycine cleavage system is loosely attached to the inner membrane of the mitochondria. Mutations in this enzymatic system are linked with glycine encephalopathy.[2]

  1. ^ Kikuchi G (June 1973). "The glycine cleavage system: composition, reaction mechanism, and physiological significance". Mol. Cell. Biochem. 1 (2): 169–87. doi:10.1007/BF01659328. PMID 4585091. S2CID 22516474.
  2. ^ a b c Kikuchi G (2008). "The glycine cleavage system: reaction mechanism, physiological significance, and hyperglycinemia". Proc. Jpn. Acad. Ser. B Phys. Biol. Sci. 84 (7): 246–63. Bibcode:2008PJAB...84..246K. doi:10.2183/pjab.84.246. PMC 3666648. PMID 18941301.
  3. ^ Douce R, Bourguignon J, Neuburger M, Rébeillé F (April 2001). "The glycine decarboxylase system: a fascinating complex". Trends Plant Sci. 6 (4): 167–76. doi:10.1016/S1360-1385(01)01892-1. PMID 11286922.

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Glycine cleavage system

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The glycine cleavage system (GCS) is also known as the glycine decarboxylase complex or GDC. The system is a series of enzymes that are triggered in response...

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Glycine encephalopathy

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metabolism. The disease is caused by defects in the glycine cleavage system, an enzyme responsible for glycine catabolism. There are several forms of the disease...

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Glycine

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mentioned above. In this context, the enzyme system involved is usually called the glycine cleavage system: Glycine + tetrahydrofolate + NAD+ ⇌ CO2 + NH+ 4...

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GCSH

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Glycine cleavage system H protein, mitochondrial (abbreviated as GCSH) is a protein that in humans is encoded by the GCSH gene. Degradation of glycine...

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Lipoyl amidotransferase

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(EC 2.3.1.200, LipL (gene)) is an enzyme with systematic name (glycine cleavage system H)-N6-lipoyl-L-lysine:(lipoyl-carrier protein)-N6-L-lysine lipoyltransferase...

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Lipoic acid

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acetyl coenzyme A. The glycine cleavage system differs from the other complexes, and has a different nomenclature. In this system, the H protein is a free...

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Glycine dehydrogenase

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name for the Glycine cleavage system P-protein (EC 1.4.4.2). or the glycine dehydroganse (cyanide forming)(EC 1.4.99.5). or the glycine dehydrogenase...

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Mitochondrion

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branched-chain α-ketoacid dehydrogenase complex (BCKDC), and in the glycine cleavage system (GCS), mtFASII has an influence on energy metabolism. Other products...

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Calvin cycle

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converted via glycolate and glyoxalate to glycine. Via the glycine cleavage system and tetrahydrofolate, two glycines are converted into serine plus CO 2....

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GCS

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Gender confirming surgery Glasgow Coma Scale Glucocorticosteroids Glycine cleavage system General Campaign Star (Canada), a Canadian Forces medal Grand Central...

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Fatty acid synthesis

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α-ketoglutarate dehydrogenase complex, the BCKDH complex and the glycine cleavage system (GCS), among others. Disorders in mtFASII lead to the following...

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Aminomethyltransferase

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CSF-to-plasma glycine ratio suggests the diagnosis. Enzymatic confirmation of the diagnosis relies on measurement of glycine cleavage system (GCS) enzyme...

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International Working Group on Neurotransmitter Related Disorders

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phosphatase, phosphoserine aminotransferase, the glycine cleavage system (the deficiency is called glycine encephalopathy or non-ketotic hyperglycinaemia)...

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GCST

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GCST can stand for: New standard tuning "Glycine cleavage system T protein", another name for aminomethyltransferase This disambiguation page lists articles...

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Paratrimastix

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Mitochondrion-Like Organelle of Trimastix pyriformis Contains the Complete Glycine Cleavage System". PLOS ONE. 8 (3): e55417. Bibcode:2013PLoSO...855417Z. doi:10...

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Paratrimastix pyriformis

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Mitochondrion-Like Organelle of Trimastix pyriformis Contains the Complete Glycine Cleavage System". PLOS ONE. 8 (3): e55417. Bibcode:2013PLoSO...855417Z. doi:10...

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Rickettsiella

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response regulator transcription factor, 30S ribosomal protein S2, glycine cleavage system aminomethyltransferase GcvT, M3 family metallopeptidase, lysm...

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2A peptides

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efficiency. The apparent cleavage is triggered by ribosomal skipping of the peptide bond between the proline (P) and glycine (G) in C-terminal of 2A peptide...

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Haladaptatus paucihalophilus

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pathway, the threonine aldolase pathways, and the reverse of the glycine cleavage system. The size of the genome of H. paucihalophilus is 4,317,540 total...

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Dihydrolipoamide dehydrogenase

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and branched-chain amino acid-dehydrogenase complexes and the glycine cleavage system, all in the mitochondrial matrix. In these complexes, DLD converts...

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Serine dehydratase

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glycine breakdown were measured. Glycine can be converted into serine and either become pyruvate via serine dehydratase or undergo oxidative cleavage...

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Proteolysis

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proteolytic cleavage into individual smaller polypeptide chains. The polyprotein pro-opiomelanocortin (POMC) contains many polypeptide hormones. The cleavage pattern...

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Diplorickettsia massiliensis

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response regulator transcription factor, 30S ribosomal protein S2, glycine cleavage system aminomethyltransferase GcvT, M3 family metallopeptidase, lysm...

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MAEB RNA motif

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with a metabolic role, and indeed a weak association with the glycine cleavage system was observed. Three hypotheses for the biological role of MAEB...

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DLST

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in rat liver mitochondria: presence of interaction between the glycine cleavage system and alpha-keto acid dehydrogenase complexes". Arch. Biochem. Biophys...

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