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Gene Review

GLDC  -  glycine dehydrogenase (decarboxylating)

Homo sapiens

Synonyms: GCE, GCSP, Glycine cleavage system P protein, Glycine decarboxylase, Glycine dehydrogenase (aminomethyl-transferring), ...
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Disease relevance of GLDC


High impact information on GLDC


Chemical compound and disease context of GLDC


Biological context of GLDC


Anatomical context of GLDC


Associations of GLDC with chemical compounds

  • Gene Symbol: GLDC. Disease: NKH glycine encephalopathy [21].
  • A single nucleotide substitution that abolishes the initiator methionine codon of the GLDC gene is prevalent among patients with glycine encephalopathy in Jerusalem [22].
  • The lipoamide arm in the glycine decarboxylase complex is not freely swinging [23].
  • The mitochondrial glycine decarboxylase multienzyme system, connected to serine hydroxymethyltransferase through a soluble pool of tetrahydrofolate, consists of four different component enzymes, the P-, H-, T- and L-proteins [9].
  • In the catalytic cycle of glycine decarboxylase, emphasis is given to the lipoate-dependent H-protein that plays a pivotal role, acting as a mobile substrate that commutes successively between the other three proteins [9].

Enzymatic interactions of GLDC

  • Therefore, it was concluded that the loss of glycine decarboxylase activity was due to an inhibition of the reaction catalyzed by T-protein, which required ATP for its activation [24].

Other interactions of GLDC


Analytical, diagnostic and therapeutic context of GLDC

  • GLDC expression in lymphoblasts was studied by Northern blot and reverse transcriptase PCR analysis [29].
  • Furthermore, we have devised a semi-quantitative PCR to estimate the number of GLDC alleles by using psiGLDC as an internal control and have confirmed the homozygosity and heterozygosity of the deletion in the patient and his parents, respectively [14].
  • METHODS: The [1-(13)C]glycine breath test was performed in 10 control subjects and 5 glycine encephalopathy patients with GLDC mutation, including 1 patient with mild glycine encephalopathy [30].
  • The glycine cleavage system. Molecular cloning of the chicken and human glycine decarboxylase cDNAs and some characteristics involved in the deduced protein structures [16].
  • Prenatal diagnosis is possible by determining the activity of GCE and also by DNA analysis [31].


  1. Treatment from birth of nonketotic hyperglycinemia due to a novel GLDC mutation. Korman, S.H., Wexler, I.D., Gutman, A., Rolland, M.O., Kanno, J., Kure, S. Ann. Neurol. (2006) [Pubmed]
  2. Glycine decarboxylase mutations: a distinctive phenotype of nonketotic hyperglycinemia in adults. Dinopoulos, A., Kure, S., Chuck, G., Sato, K., Gilbert, D.L., Matsubara, Y., Degrauw, T. Neurology (2005) [Pubmed]
  3. Purification, characterization and function of dihydrolipoamide dehydrogenase from the cyanobacterium Anabaena sp. strain P.C.C. 7119. Serrano, A. Biochem. J. (1992) [Pubmed]
  4. Mitochondrial oxidative burst involved in apoptotic response in oats. Yao, N., Tada, Y., Sakamoto, M., Nakayashiki, H., Park, P., Tosa, Y., Mayama, S. Plant J. (2002) [Pubmed]
  5. The pathogen-inducible nitric oxide synthase (iNOS) in plants is a variant of the P protein of the glycine decarboxylase complex. Klessig, D.F., Ytterberg, A.J., van Wijk, K.J. Cell (2004) [Pubmed]
  6. Defective glycine cleavage system in nonketotic hyperglycinemia. Occurrence of a less active glycine decarboxylase and an abnormal aminomethyl carrier protein. Hiraga, K., Kochi, H., Hayasaka, K., Kikuchi, G., Nyhan, W.L. J. Clin. Invest. (1981) [Pubmed]
  7. Victorin induction of an apoptotic/senescence-like response in oats. Navarre, D.A., Wolpert, T.J. Plant Cell (1999) [Pubmed]
  8. Identification of the 100-kD victorin binding protein from oats. Wolpert, T.J., Navarre, D.A., Moore, D.L., Macko, V. Plant Cell (1994) [Pubmed]
  9. The glycine decarboxylase system: a fascinating complex. Douce, R., Bourguignon, J., Neuburger, M., Rébeillé, F. Trends Plant Sci. (2001) [Pubmed]
  10. Persistent NKH with transient or absent symptoms and a homozygous GLDC mutation. Korman, S.H., Boneh, A., Ichinohe, A., Kojima, K., Sato, K., Ergaz, Z., Gomori, J.M., Gutman, A., Kure, S. Ann. Neurol. (2004) [Pubmed]
  11. Molecular characterization of FOX-4, a new AmpC-type plasmid-mediated beta-lactamase from an Escherichia coli strain isolated in Spain. Bou, G., Oliver, A., Ojeda, M., Monzón, C., Martínez-Beltrán, J. Antimicrob. Agents Chemother. (2000) [Pubmed]
  12. Isolation of an atypically small lipoamide dehydrogenase involved in the glycine decarboxylase complex from Eubacterium acidaminophilum. Freudenberg, W., Dietrichs, D., Lebertz, H., Andreesen, J.R. J. Bacteriol. (1989) [Pubmed]
  13. Granulocyte elastase activity and PGE2 levels in gingival crevicular fluid in relation to the presence of subgingival periodontopathogens in subjects with untreated adult periodontitis. Jin, L.J., Söder, P.O., Leung, W.K., Corbet, E.F., Samaranayake, L.P., Söder, B., Davies, W.I. Journal of clinical periodontology. (1999) [Pubmed]
  14. Human glycine decarboxylase gene (GLDC) and its highly conserved processed pseudogene (psiGLDC): their structure and expression, and the identification of a large deletion in a family with nonketotic hyperglycinemia. Takayanagi, M., Kure, S., Sakata, Y., Kurihara, Y., Ohya, Y., Kajita, M., Tada, K., Matsubara, Y., Narisawa, K. Hum. Genet. (2000) [Pubmed]
  15. Comprehensive mutation analysis of GLDC, AMT, and GCSH in nonketotic hyperglycinemia. Kure, S., Kato, K., Dinopoulos, A., Gail, C., DeGrauw, T.J., Christodoulou, J., Bzduch, V., Kalmanchey, R., Fekete, G., Trojovsky, A., Plecko, B., Breningstall, G., Tohyama, J., Aoki, Y., Matsubara, Y. Hum. Mutat. (2006) [Pubmed]
  16. The glycine cleavage system. Molecular cloning of the chicken and human glycine decarboxylase cDNAs and some characteristics involved in the deduced protein structures. Kume, A., Koyata, H., Sakakibara, T., Ishiguro, Y., Kure, S., Hiraga, K. J. Biol. Chem. (1991) [Pubmed]
  17. Glycine decarboxylase and pyruvate dehydrogenase complexes share the same dihydrolipoamide dehydrogenase in pea leaf mitochondria: evidence from mass spectrometry and primary-structure analysis. Bourguignon, J., Merand, V., Rawsthorne, S., Forest, E., Douce, R. Biochem. J. (1996) [Pubmed]
  18. Purification and properties of glycine decarboxylase, a component of the glycine cleavage system, from rat liver mitochondria and immunochemical comparison of this enzyme from various sources. Hayasaka, K., Kochi, H., Hiraga, K., Kikuchi, G. J. Biochem. (1980) [Pubmed]
  19. Electrochemical immunoassay of membrane P-glycoprotein by immobilization of cells on gold nanoparticles modified on a methoxysilyl-terminated butyrylchitosan matrix. Du, D., Ju, H., Zhang, X., Chen, J., Cai, J., Chen, H. Biochemistry (2005) [Pubmed]
  20. Structural and biochemical bases of photorespiration in C4 plants: quantification of organelles and glycine decarboxylase. Yoshimura, Y., Kubota, F., Ueno, O. Planta (2004) [Pubmed]
  21. Gene Symbol: GLDC. Disease: NKH glycine encephalopathy. Toone, J.R., Applegarth, D.A., Laliberte, G. Hum. Genet. (2003) [Pubmed]
  22. A single nucleotide substitution that abolishes the initiator methionine codon of the GLDC gene is prevalent among patients with glycine encephalopathy in Jerusalem. Boneh, A., Korman, S.H., Sato, K., Kanno, J., Matsubara, Y., Lerer, I., Ben-Neriah, Z., Kure, S. J. Hum. Genet. (2005) [Pubmed]
  23. The lipoamide arm in the glycine decarboxylase complex is not freely swinging. Cohen-Addad, C., Pares, S., Sieker, L., Neuburger, M., Douce, R. Nat. Struct. Biol. (1995) [Pubmed]
  24. Activation of glycine decarboxylase in pea leaf mitochondria by ATP. Zhang, Q., Wiskich, J.T. Arch. Biochem. Biophys. (1995) [Pubmed]
  25. Heterozygous GLDC and GCSH gene mutations in transient neonatal hyperglycinemia. Kure, S., Kojima, K., Ichinohe, A., Maeda, T., Kalmanchey, R., Fekete, G., Berg, S.Z., Filiano, J., Aoki, Y., Suzuki, Y., Izumi, T., Matsubara, Y. Ann. Neurol. (2002) [Pubmed]
  26. Detection of mutations in the glycine decarboxylase gene in patients with nonketotic hyperglycinaemia. Sellner, L., Edkins, E., Greed, L., Lewis, B. Mol. Genet. Metab. (2005) [Pubmed]
  27. 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). Toone, J.R., Applegarth, D.A., Coulter-Mackie, M.B., James, E.R. Mol. Genet. Metab. (2001) [Pubmed]
  28. Biochemical dissection of photorespiration. Douce, R., Neuburger, M. Curr. Opin. Plant Biol. (1999) [Pubmed]
  29. Mild glycine encephalopathy (NKH) in a large kindred due to a silent exonic GLDC splice mutation. Flusser, H., Korman, S.H., Sato, K., Matsubara, Y., Galil, A., Kure, S. Neurology (2005) [Pubmed]
  30. Rapid diagnosis of glycine encephalopathy by (13)C-glycine breath test. Kure, S., Korman, S.H., Kanno, J., Narisawa, A., Kubota, M., Takayanagi, T., Takayanagi, M., Saito, T., Matsui, A., Kamada, F., Aoki, Y., Ohura, T., Matsubara, Y. Ann. Neurol. (2006) [Pubmed]
  31. Non-ketotic hyperglycinemia: a life-threatening disorder in the neonate. Tada, K., Kure, S., Takayanagi, M., Kume, A., Narisawa, K. Early Hum. Dev. (1992) [Pubmed]
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