Enzyme complexity in intermediary metabolism

Journal of Inherited Metabolic Disease

Volumn 38, Issue 4, 2015, Pages 721-727

  Van Schaftingen, Emile ^a   Veiga da Cunha, Maria ^a   Linster, Carole L ^b  

^a DE DUVE INSTITUTE   (Belgium)

^b UNIVERSITY OF LUXEMBOURG   (Luxembourg)

Author keywords

[No Author keywords available]

Indexed keywords

CRYSTALLIN; EPIMERASE; FATTY ACID SYNTHASE; FRUCTOSE BISPHOSPHATE ALDOLASE; GDP MANNOSE PYROPHOSPHORYLASE A; GLUCOKINASE; GLUCOSE 6 PHOSPHATE ISOMERASE; GLUTAMINE FRUCTOSE 6 PHOSPHATE AMINOTRANSFERASE; HYDROLYASE; IRON REGULATORY PROTEIN 1; L 2 HYDROXYGLUTARATE DEHYDROGENASE; MULTIFUNCTIONAL ENZYME; NAD(P)HX DEHYDRATASE; NAD(P)HX EPIMERASE; OXIDOREDUCTASE; PYRUVATE KINASE; PYRUVATE KINASE MUSCLE 2; RNA; UNCLASSIFIED DRUG; ENZYME; MULTIENZYME COMPLEX;

ALLOSTERISM; ALTERNATIVE RNA SPLICING; ARTICLE; BINDING SITE; CATALYSIS; ENZYME ACTIVE SITE; ENZYME ACTIVITY; ENZYME PHOSPHORYLATION; ENZYME SPECIFICITY; ENZYME SUBSTRATE COMPLEX; HUMAN; METABOLIC REGULATION; METABOLITE; METABOLOME; MUTATION; OXIDATION REDUCTION REACTION; PROTEIN DOMAIN; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN RNA BINDING; SECRETORY PATHWAY; ANIMAL; GENETICS; METABOLISM;

ANIMALS; ENZYMES; HUMANS; METABOLISM; MULTIENZYME COMPLEXES; MULTIFUNCTIONAL ENZYMES; SUBSTRATE SPECIFICITY;

EID: 84937252587     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-015-9821-0     Document Type: Article

References (41)

1. Adler LN, Gomez TA, Clarke SG, Linster CL (2011) A novel GDP-D-glucose phosphorylase involved in quality control of the nucleoside diphosphate sugar pool in Caenorhabditis elegans and mammals. J Biol Chem 286:21511–21523
2. Ando T, Rasmussen K, Wright JM, Nyhan WL (1972) Isolation and identification of methylcitrate, a major metabolic product of propionate in patients with propionic acidemia. J Biol Chem 247:2200–2204
3. Asipu A, Hayward BE, O’Reilly J, Bonthron DT (2003) Properties of normal and mutant recombinant human ketohexokinases and implications for the pathogenesis of essential fructosuria. Diabetes 52:2426–2432
4. Beadle GW, Tatum EL (1941) Genetic control of biochemical reactions in neurospora. Proc Natl Acad Sci U S A 27:499–506
5. Beld J, Lee DJ, Burkart MD (2015) Fatty acid biosynthesis revisited: structure elucidation and metabolic engineering. Mol BioSyst 11:38–59
6. Camacho JA, Obie C, Biery B et al (1999) Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter. Nat Genet 22:151–158
7. Carss KJ, Stevens E, Foley AR et al (2013) Mutations in GDP-mannose pyrophosphorylase B cause congenital and limb-girdle muscular dystrophies associated with hypoglycosylation of α-dystroglycan. Am J Hum Genet 93:29–41
8. Cieśla J (2006) Metabolic enzymes that bind RNA: yet another level of cellular regulatory network? Acta Biochim Pol 53:11–32
9. Copley SD (2012) Moonlighting is mainstream: paradigm adjustment required. Bioessays 34:578–588
10. Dang L, White DW, Gross S et al (2009) Cancer-associated IDH1 mutations produce 2-hydroxyglutarate. Nature 462:739–744
11. Fairbank M, St-Pierre P, Nabi IR (2009) The complex biology of autocrine motility factor/phosphoglucose isomerase (AMF/PGI) and its receptor, the gp78/AMFR E3 ubiquitin ligase. Mol BioSyst 5:793–801
12. Fiehn O, Barupal DK, Kind T (2011) Extending biochemical databases by metabolomic surveys. J Biol Chem 286:23637–23643
13. Gitzelmann R, Steinmann B, Van Den Berghe G (1989) Disorders of fructose metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis of inherited disease, 6th edn. McGraw-Hill, New York, pp 399–424
14. Hanson AD, Pribat A, Waller JC, de Crécy-Lagard V (2009) ‘Unknown’ proteins and ‘orphan’ enzymes: the missing half of the engineering parts list- and how to find it. Biochem J 425:1–11
15. Iqbal MA, Gupta V, Gopinath P, Mazurek S, Bamezai RN (2014) Pyruvate kinase M2 and cancer: an updated assessment. FEBS Lett 588:2685–2692
16. Iyanagi T (2007) Molecular mechanism of phase I and phase II drug-metabolizing enzymes: implications for detoxification. Int Rev Cytol 260:35–112
17. Jeffery CJ (1999) Moonlighting proteins. Trends Biochem Sci 24:8–11
18. Jeffery CJ (2003) Moonlighting proteins: old proteins learning new tricks. Trends Genet 19:415–417
19. Jeffery CJ (2011) Proteins with neomorphic moonlighting functions in disease. IUBMB Life 63:489–494
20. Kemp RG, Gunasekera D (2002) Evolution of the allosteric ligand sites of mammalian phosphofructo-1-kinase. Biochemistry 41:9426–9430
21. Koehler K, Malik M, Mahmood S et al (2013) Mutations in GMPPA cause a glycosylation disorder characterized by intellectual disability and autonomic dysfunction. Am J Hum Genet 93:727–734
22. Kugler W, Breme K, Laspe P et al (1998) Molecular basis of neurological dysfunction coupled with haemolytic anaemia in human glucose-6-phosphate isomerase (GPI) deficiency. Hum Genet 103:450–454
23. Linster CL, Noël G, Stroobant V et al (2011) Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading. J Biol Chem 286:42992–43003
24. Linster CL, Van Schaftingen E, Hanson AD (2013) Metabolite damage and its repair or pre-emption. Nat Chem Biol 9:72–80
25. Marbaix AY, Noël G, Detroux AM, Vertommen D, Van Schaftingen E, Linster CL (2011) Extremely conserved ATP- or ADP-dependent enzymatic system for nicotinamide nucleotide repair. J Biol Chem 286:41246–41252
26. Marbaix AY, Tyteca D, Niehaus TD, Hanson AD, Linster CL, Van Schaftingen E (2014) Occurrence and subcellular distribution of the NADPHX repair system in mammals. Biochem J 460:49–58
27. Middleton RJ (1990) Hexokinases and glucokinases. Biochem Soc Trans 18:180–183
28. Paravicini G, Kretschmer M (1992) The yeast FBP26 gene codes for a fructose-2,6-bisphosphatase. Biochemistry 31:7126–7133
29. Rider MH, Bertrand L, Vertommen D, Michels PA, Rousseau GG, Hue L (2004) 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase: head-to-head with a bifunctional enzyme that controls glycolysis. Biochem J 381:561–579
30. Shinabarger D, Berry A, May TB, Rothmel R, Fialho A, Chakrabarty AM (1991) Purification and characterization of phosphomannose isomerase-guanosine diphospho-D-mannose pyrophosphorylase. A bifunctional enzyme in the alginate biosynthetic pathway of Pseudomonas aeruginosa. J Biol Chem 266:2080–2088
31. Tait SW, Green DR (2010) Mitochondria and cell death: outer membrane permeabilization and beyond. Nat Rev Mol Cell Biol 11:621–632
32. Teplyakov A, Obmolova G, Badet B, Badet-Denisot MA (2001) Channeling of ammonia in glucosamine-6-phosphate synthase. J Mol Biol 313:1093–1102
33. Van Schaftingen E, Detheux M, Veiga da Cunha M (1994) Short-term control of glucokinase activity: role of a regulatory protein. FASEB J 8:414–419
34. Van Schaftingen E, Rzem R, Veiga-da-Cunha M (2009) L-2-Hydroxyglutaric aciduria, a disorder of metabolite repair. J Inherit Metab Dis 32:135–142
35. Van Schaftingen E, Rzem R, Marbaix A, Collard F, Veiga-da-Cunha M, Linster CL (2013) Metabolite proofreading, a neglected aspect of intermediary metabolism. J Inherit Metab Dis 36:427–434
36. Veiga-da-Cunha M, Sokolova T, Opperdoes F, Van Schaftingen E (2009) Evolution of vertebrate glucokinase regulatory protein from a bacterial N-acetylmuramate 6-phosphate etherase. Biochem J 423:323–332
37. Veiga-da-Cunha M, Hadi F, Balligand T, Stroobant V, Van Schaftingen E (2012) Molecular identification of hydroxylysine kinase and of ammoniophospholyases acting on 5-phosphohydroxy-L-lysine and phosphoethanolamine. J Biol Chem 287:7246–7255
38. Veiga-da-Cunha M, Chevalier N, Stroobant V, Vertommen D, Van Schaftingen E (2014) Metabolite proofreading in carnosine and homocarnosine synthesis: molecular identification of PM20D2 as β-alanyl-lysine dipeptidase. J Biol Chem 289:19726–19736
39. Weil-Malherbe H (1937) The oxidation of l(−)α-hydroxyglutaric acid in animal tissues. Biochem J 31:2080–2094
40. Wistow G (2012) The human crystallin gene families. Hum Genomics 6:26
41. Xue HH, Sakaguchi T, Fujie M, Ogawa H, Ichiyama A (1999) Flux of the L-serine metabolism in rabbit, human, and dog livers. Substantial contributions of both mitochondrial and peroxisomal serine:pyruvate/alanine:glyoxylate aminotransferase. J Biol Chem 274:16028–16033