Carboxylases and decarboxylases
Jump toCarboxylases
The carboxylases allow the production of new carbon-carbon bonds by introducing HCO3- or CO2 into target molecules. Two groups of carboxylase activities, some of which are bidirectional, can be defined on the basis of the cofactor requirement, making use of biotin (EC 6.4.1.-) or vitamin K hydroquinone (EC 4.1.1.-).
Unless otherwise stated all data refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).
Enzymes 
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Pyruvate carboxylase Show »« Hide
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Acetyl-CoA carboxylase 1 Show »« Hide
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Acetyl-CoA carboxylase 2 Show »« Hide
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Propionyl-CoA carboxylase Show »« Hide
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γ-Glutamyl carboxylase Show »« Hide
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Subunits
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Propionyl-CoA carboxylase α subunit Show »« Hide
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Propionyl-CoA carboxylase β subunit Show »« Hide
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Dicarboxylic acids including citric acid are able to activate ACC1/ACC2 activity allosterically. PCC is able to function in forward and reverse modes as a ligase (carboxylase) or lyase (decarboxylase) activity, respectively. Loss-of-function mutations in GGCX are associated with clotting disorders.
Decarboxylases
The decarboxylases generate CO2 and the indicated products from acidic substrates, requiring pyridoxal phosphate (ADC, AADC, GAD, HDC, ODC and PSDC) or pyruvic acid (SAMDC and PSDC) as a co-factor.
Unless otherwise stated all data refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).
Enzymes
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S-Adenosylmethionine decarboxylase Show »« Hide
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L-Arginine decarboxylase Show »« Hide
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L-Aromatic amino-acid decarboxylase Show »« Hide
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Glutamic acid decarboxylase 1 Show »« Hide
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Glutamic acid decarboxylase 2 Show »« Hide
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Histidine decarboxylase Show »« Hide
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Malonyl-CoA decarboxylase Show »« Hide
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Ornithine decarboxylase Show »« Hide
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Phosphatidylserine decarboxylase Show »« Hide
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Akbarian, S; Huang, HS. (2006) Molecular and cellular mechanisms of altered GAD1/GAD67 expression in schizophrenia and related disorders. Brain Res Rev, 52 (2): 293-304. [PMID:16759710]
Bale, S; Ealick, SE. (2010) Structural biology of S-adenosylmethionine decarboxylase. Amino Acids, 38 (2): 451-60. [PMID:19997761]
Brownsey, RW; Boone, AN; Elliott, JE; Kulpa, JE; Lee, WM. (2006) Regulation of acetyl-CoA carboxylase. Biochem. Soc. Trans., 34 (Pt 2): 223-7. [PMID:16545081]
Elmets, CA; Athar, M. (2010) Targeting ornithine decarboxylase for the prevention of nonmelanoma skin cancer in humans. Cancer Prev Res (Phila), 3 (1): 8-11. [PMID:20051367]
Jitrapakdee, S; St Maurice, M; Rayment, I; Cleland, WW; Wallace, JC; Attwood, PV. (2008) Structure, mechanism and regulation of pyruvate carboxylase. Biochem. J., 413 (3): 369-87. [PMID:18613815]
Jitrapakdee, S; Vidal-Puig, A; Wallace, JC. (2006) Anaplerotic roles of pyruvate carboxylase in mammalian tissues. Cell. Mol. Life Sci., 63 (7-8): 843-54. [PMID:16505973]
Marin-Valencia, I; Roe, CR; Pascual, JM. (2010) Pyruvate carboxylase deficiency: mechanisms, mimics and anaplerosis. Mol. Genet. Metab., 101 (1): 9-17. [PMID:20598931]
Moya-García, AA; Pino-Angeles, A; Gil-Redondo, R; Morreale, A; Sánchez-Jiménez, F. (2009) Structural features of mammalian histidine decarboxylase reveal the basis for specific inhibition. Br. J. Pharmacol., 157 (1): 4-13. [PMID:19413567]
Pegg, AE. (2006) Regulation of ornithine decarboxylase. J. Biol. Chem., 281 (21): 14529-32. [PMID:16459331]
Pegg, AE. (2009) S-Adenosylmethionine decarboxylase. Essays Biochem., 46: 25-45. [PMID:20095968]
Saggerson, D. (2008) Malonyl-CoA, a key signaling molecule in mammalian cells. Annu. Rev. Nutr., 28: 253-72. [PMID:18598135]
Smith, KJ; Skelton, H. (2006) alpha-Difluoromethylornithine, a polyamine inhibitor: its potential role in controlling hair growth and in cancer treatment and chemo-prevention. Int. J. Dermatol., 45 (4): 337-44. [PMID:16650154]
Tong, L; Harwood, HJ. (2006) Acetyl-coenzyme A carboxylases: versatile targets for drug discovery. J. Cell. Biochem., 99 (6): 1476-88. [PMID:16983687]
Wolfgang, MJ; Lane, MD. (2006) The role of hypothalamic malonyl-CoA in energy homeostasis. J. Biol. Chem., 281 (49): 37265-9. [PMID:17018521]
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