Gene Symbol: Ddc
Description: dopa decarboxylase
Alias: Aadc, aromatic-L-amino-acid decarboxylase
Species: mouse
Products:     Ddc

Top Publications

  1. Smidt M, Smits S, Bouwmeester H, Hamers F, van der Linden A, Hellemons A, et al. Early developmental failure of substantia nigra dopamine neurons in mice lacking the homeodomain gene Pitx3. Development. 2004;131:1145-55 pubmed
    ..However, an overall lower activity was observed. The results suggest that Pitx3 is specifically required for the formation of the SNc subfield at the onset of dopaminergic neuron differentiation...
  2. Andersson E, Jensen J, Parmar M, Guillemot F, Bjorklund A. Development of the mesencephalic dopaminergic neuron system is compromised in the absence of neurogenin 2. Development. 2006;133:507-16 pubmed
  3. Hendricks T, Fyodorov D, Wegman L, Lelutiu N, Pehek E, Yamamoto B, et al. Pet-1 ETS gene plays a critical role in 5-HT neuron development and is required for normal anxiety-like and aggressive behavior. Neuron. 2003;37:233-47 pubmed
    ..These findings indicate that Pet-1 is a critical determinant of 5-HT neuron identity and implicate a Pet-1-dependent program in serotonergic modulation of behavior. ..
  4. Zhang M, Yao B, Wang S, Fan X, Wu G, Yang H, et al. Intrarenal dopamine deficiency leads to hypertension and decreased longevity in mice. J Clin Invest. 2011;121:2845-54 pubmed publisher
    ..Although the kidney synthesizes dopamine through the actions of aromatic amino acid decarboxylase (AADC) in the proximal tubule, previous studies have not discriminated between the roles of extrarenal and intrarenal ..
  5. Jacobs F, Smits S, Noorlander C, von Oerthel L, van der Linden A, Burbach J, et al. Retinoic acid counteracts developmental defects in the substantia nigra caused by Pitx3 deficiency. Development. 2007;134:2673-84 pubmed
    ..Here, we propose a novel mechanism in which RA is involved in mdDA neuronal development and maintenance, providing new insights into subset-specific vulnerability in PD. ..
  6. Ferri A, Lin W, Mavromatakis Y, Wang J, Sasaki H, Whitsett J, et al. Foxa1 and Foxa2 regulate multiple phases of midbrain dopaminergic neuron development in a dosage-dependent manner. Development. 2007;134:2761-9 pubmed
    ..Altogether, our results demonstrate that Foxa1 and Foxa2 regulate multiple phases of midbrain dopaminergic neuron development in a dosage-dependent manner. ..
  7. Jacob J, Storm R, Castro D, Milton C, Pla P, Guillemot F, et al. Insm1 (IA-1) is an essential component of the regulatory network that specifies monoaminergic neuronal phenotypes in the vertebrate hindbrain. Development. 2009;136:2477-85 pubmed publisher
    ..Thus, Insm1 regulates the synthesis of distinct monoaminergic neurotransmitters by acting combinatorially with, or independently of, Ascl1 in specific monoaminergic populations. ..
  8. Smits S, Ponnio T, Conneely O, Burbach J, Smidt M. Involvement of Nurr1 in specifying the neurotransmitter identity of ventral midbrain dopaminergic neurons. Eur J Neurosci. 2003;18:1731-8 pubmed
    ..dopamine transporter (DAT), the vesicular monoamine transporter 2 (VMAT2) and l-aromatic amino acid decarboxylase (AADC) in addition to TH specifically in mesDA neurons...
  9. Wu H, Choi S, Levitt P. Differential patterning of genes involved in serotonin metabolism and transport in extra-embryonic tissues of the mouse. Placenta. 2016;42:74-83 pubmed publisher
    ..Mid-through late gestation expression of 5-HT system-related enzymes, Tph1, Ddc, Maoa, and 5-HT transporters, Sert/Slc6a4, Oct3/Slc22a3, Vmat2/Slc18a2, and 5-HT in placenta and yolk sac were ..

More Information


  1. Shiura H, Nakamura K, Hikichi T, Hino T, Oda K, Suzuki Migishima R, et al. Paternal deletion of Meg1/Grb10 DMR causes maternalization of the Meg1/Grb10 cluster in mouse proximal Chromosome 11 leading to severe pre- and postnatal growth retardation. Hum Mol Genet. 2009;18:1424-38 pubmed publisher
    ..It was found that Cobl and Ddc, the neighboring genes of Meg1/Grb10, also comprise the imprinted region...
  2. Hoerder Suabedissen A, Wang W, Lee S, Davies K, Goffinet A, Rakić S, et al. Novel markers reveal subpopulations of subplate neurons in the murine cerebral cortex. Cereb Cortex. 2009;19:1738-50 pubmed publisher
    ..These new markers open up possibilities for further identification of subplate subpopulations in research and in neuropathological diagnosis. ..
  3. Hoekstra E, von Oerthel L, van der Heide L, Kouwenhoven W, Veenvliet J, Wever I, et al. Lmx1a encodes a rostral set of mesodiencephalic dopaminergic neurons marked by the Wnt/B-catenin signaling activator R-spondin 2. PLoS ONE. 2013;8:e74049 pubmed publisher
  4. Yang S, Yao B, Zhou Y, Yin H, Zhang M, Harris R. Intrarenal dopamine modulates progressive angiotensin II-mediated renal injury. Am J Physiol Renal Physiol. 2012;302:F742-9 pubmed publisher
    ..In aromatic l-amino acid decarboxylase knockout (AADC KO) mice, a model of intrarenal dopamine deficiency due to selective proximal tubule AADC deletion, which inhibits ..
  5. Eaton M, Gudehithlu K, Quach T, Silvia C, Hadjiconstantinou M, Neff N. Distribution of aromatic L-amino acid decarboxylase mRNA in mouse brain by in situ hybridization histology. J Comp Neurol. 1993;337:640-54 pubmed
    ..The presence of AAAD message in these neurons suggests that the enzyme has functions other than that for the synthesis of the classical biogenic amine neurotransmitters. ..
  6. Garfield A, Cowley M, Smith F, Moorwood K, Stewart Cox J, Gilroy K, et al. Distinct physiological and behavioural functions for parental alleles of imprinted Grb10. Nature. 2011;469:534-8 pubmed publisher
    ..Thus Grb10 is, so far, a unique imprinted gene, able to influence distinct physiological processes, fetal growth and adult behaviour, owing to actions of the two parental alleles in different tissues. ..
  7. Seta Y, Oda M, Kataoka S, Toyono T, Toyoshima K. Mash1 is required for the differentiation of AADC-positive type III cells in mouse taste buds. Dev Dyn. 2011;240:775-84 pubmed publisher
    ..We found that aromatic L-amino acid decarboxylase (AADC) cells were not evident in either the circumvallate papilla epithelia or in taste buds in the soft palates of Mash1 ..
  8. Ratzka A, Baron O, Stachowiak M, Grothe C. Fibroblast growth factor 2 regulates dopaminergic neuron development in vivo. J Neurochem. 2012;122:94-105 pubmed publisher
    ..Increased levels of nuclear FgfR1 in E14.5 FGF-2-deficient mice suggest alterations of integrative nuclear FgfR1 signaling (INFS). In summary, FGF-2 restricts SNpc DA neurogenesis in vivo during late stages of embryonic development. ..
  9. Sgadò P, Ferretti E, Grbec D, Bozzi Y, Simon H. The atypical homeoprotein Pbx1a participates in the axonal pathfinding of mesencephalic dopaminergic neurons. Neural Dev. 2012;7:24 pubmed publisher
  10. Kubovcakova L, Krizanova O, Kvetnansky R. Identification of the aromatic L-amino acid decarboxylase gene expression in various mice tissues and its modulation by immobilization stress in stellate ganglia. Neuroscience. 2004;126:375-80 pubmed
    ..immunoreactive protein has been intensively studied, less is known about the aromatic L-amino acid decarboxylase (AADC), the enzyme, which catalyzes decarboxylation of L-dihydroxyphenylalanine to dopamine...
  11. Zhang M, Wang Y, Yao B, Gewin L, Wei S, Capdevila J, et al. Role of epoxyeicosatrienoic acids (EETs) in mediation of dopamine's effects in the kidney. Am J Physiol Renal Physiol. 2013;305:F1680-6 pubmed publisher
    ..These studies demonstrate that renal EET levels are maintained by intrarenal dopamine, and Cyp2c44-derived EETs play an important role in intrarenal dopamine-induced natriuresis and diuresis. ..
  12. Lee N, Shieh Y, Chien Y, Tzen K, Yu I, Chen P, et al. Regulation of the dopaminergic system in a murine model of aromatic L-amino acid decarboxylase deficiency. Neurobiol Dis. 2013;52:177-90 pubmed publisher
    Aromatic l-amino acid decarboxylase (AADC) is responsible for the syntheses of dopamine and serotonin. Children with AADC deficiency exhibit compromised development, particularly with regard to their motor functions...
  13. Sizova D, Velazquez H, Sampaio Maia B, Quelhas Santos J, Pestana M, Desir G. Renalase regulates renal dopamine and phosphate metabolism. Am J Physiol Renal Physiol. 2013;305:F839-44 pubmed publisher
    ..The signal to increase renal DA synthesis is strong since it overcomes a compensatory increase in COMT activity. ..
  14. Kim J, Kim A, Zhao Z, Liu X, Chen Z. Postnatal maintenance of the 5-Ht1a-Pet1 autoregulatory loop by serotonin in the raphe nuclei of the brainstem. Mol Brain. 2014;7:48 pubmed publisher
    ..Our results are relevant to understanding the pathophysiology of certain psychiatric and developmental disorders. ..
  15. Liu C, Maejima T, Wyler S, Casadesus G, Herlitze S, Deneris E. Pet-1 is required across different stages of life to regulate serotonergic function. Nat Neurosci. 2010;13:1190-8 pubmed publisher
    ..These findings indicate that Pet-1 is required across the lifespan of the mouse and that behavioral pathogenesis can result from both developmental and adult-onset alterations in serotonergic transcription. ..
  16. Menheniott T, Woodfine K, Schulz R, Wood A, Monk D, Giraud A, et al. Genomic imprinting of Dopa decarboxylase in heart and reciprocal allelic expression with neighboring Grb10. Mol Cell Biol. 2008;28:386-96 pubmed
    ..screen with mouse uniparental duplications, we have identified a novel imprinted gene, Dopa decarboxylase (Ddc), on chromosome 11...
  17. Seta Y, Kataoka S, Toyono T, Toyoshima K. Immunohistochemical localization of aromatic L-amino acid decarboxylase in mouse taste buds and developing taste papillae. Histochem Cell Biol. 2007;127:415-22 pubmed
    Aromatic L-amino acid decarboxylase (AADC) catalyses the decarboxylation of all aromatic L-amino acids...
  18. Lee N, Lee Y, Chen P, Byrne B, Hwu W. Mutation-adapted U1 snRNA corrects a splicing error of the dopa decarboxylase gene. Hum Mol Genet. 2016;25:5142-5147 pubmed publisher
    Aromatic l-amino acid decarboxylase (AADC) deficiency is an inborn error of monoamine neurotransmitter synthesis, which results in dopamine, serotonin, epinephrine and norepinephrine deficiencies...
  19. Stott S, Metzakopian E, Lin W, Kaestner K, Hen R, Ang S. Foxa1 and foxa2 are required for the maintenance of dopaminergic properties in ventral midbrain neurons at late embryonic stages. J Neurosci. 2013;33:8022-34 pubmed publisher
    ..These results extend previous findings and demonstrate a later role for Foxa genes in regulating the maintenance of dopaminergic phenotype in mDA neurons. ..
  20. Havrda M, Paolella B, Ward N, Holroyd K. Behavioral abnormalities and Parkinson's-like histological changes resulting from Id2 inactivation in mice. Dis Model Mech. 2013;6:819-27 pubmed publisher
    ..These findings document a novel role for Id2 in the maintenance of midbrain dopamine neurons. The Id2(-/-) mouse should provide unique opportunities to study the progression of neurodegenerative disorders involving the dopamine system. ..
  21. Gazea M, Tasouri E, Tolve M, Bosch V, Kabanova A, Gojak C, et al. Primary cilia are critical for Sonic hedgehog-mediated dopaminergic neurogenesis in the embryonic midbrain. Dev Biol. 2016;409:55-71 pubmed publisher
  22. Jacobs F, van Erp S, van der Linden A, von Oerthel L, Burbach J, Smidt M. Pitx3 potentiates Nurr1 in dopamine neuron terminal differentiation through release of SMRT-mediated repression. Development. 2009;136:531-40 pubmed publisher
  23. Voigt A, Hübner S, Lossow K, Hermans Borgmeyer I, Boehm U, Meyerhof W. Genetic labeling of Tas1r1 and Tas2r131 taste receptor cells in mice. Chem Senses. 2012;37:897-911 pubmed publisher
  24. Bruneau G, Thibault J, Gros F, Mattei M. Mapping of the dopa decarboxylase gene to the 11A band of the murine genome. Biochem Biophys Res Commun. 1992;186:926-30 pubmed
    A human DOPA decarboxylase (DDC) cDNA probe of 747 base pairs has been used to map the DDC gene by in situ hybridization on mouse metaphase chromosomes...
  25. Nishiguchi Y, Ohmoto M, Koki J, Enomoto T, Kominami R, Matsumoto I, et al. Bcl11b/Ctip2 is required for development of lingual papillae in mice. Dev Biol. 2016;416:98-110 pubmed publisher
    ..These results indicate that Bcl11b regulates the differentiation of keratinocytes in the tongue and identify Bcl11b as an essential factor for the lingual papilla morphogenesis. ..
  26. Cases O, Lebrand C, Giros B, Vitalis T, De Maeyer E, Caron M, et al. Plasma membrane transporters of serotonin, dopamine, and norepinephrine mediate serotonin accumulation in atypical locations in the developing brain of monoamine oxidase A knock-outs. J Neurosci. 1998;18:6914-27 pubmed
    ..This points to neuron populations that form highly precise projection maps that could be affected by 5-HT during specific developmental stages. ..
  27. Zhang M, Yao B, Yang S, Yang H, Wang S, Fan X, et al. Intrarenal dopamine inhibits progression of diabetic nephropathy. Diabetes. 2012;61:2575-84 pubmed
  28. Vassort C, Riviere M, Bruneau G, Gros F, Thibault J, Levan G, et al. Assignment of the rat genes coding for dopa decarboxylase (DDC) and glutamic acid decarboxylases (GAD1 and GAD2). Mamm Genome. 1993;4:202-6 pubmed
    ..the genes encoding three pyridoxal 5'-phosphate (PLP)-dependent decarboxylases--namely, DOPA-decarboxylase (Ddc), glutamic acid decarboxylase 1 and 2 (Gad1 and Gad2)--were assigned to rat Chromosomes (Chrs) 14, 3, and 17, ..
  29. Kadkhodaei B, Ito T, Joodmardi E, Mattsson B, Rouillard C, Carta M, et al. Nurr1 is required for maintenance of maturing and adult midbrain dopamine neurons. J Neurosci. 2009;29:15923-32 pubmed publisher
  30. Ryu S, Mahler J, Acampora D, Holzschuh J, Erhardt S, Omodei D, et al. Orthopedia homeodomain protein is essential for diencephalic dopaminergic neuron development. Curr Biol. 2007;17:873-80 pubmed
    ..Thus, Otp is one of the few known transcription factors that can determine aspects of the dopaminergic phenotype and the first known factor to control the development of the diencephalospinal dopaminergic system. ..
  31. Thuret S, Alavian K, Gassmann M, Lloyd C, Smits S, Smidt M, et al. The neuregulin receptor, ErbB4, is not required for normal development and adult maintenance of the substantia nigra pars compacta. J Neurochem. 2004;91:1302-11 pubmed
    ..Finally, an assessment of their motor function revealed no behavioral deficits. The apparent lack of any mutant phenotype suggests the presence of a strong compensatory mechanism. ..
  32. Jiang H, Xiang M. Subtype specification of GABAergic amacrine cells by the orphan nuclear receptor Nr4a2/Nurr1. J Neurosci. 2009;29:10449-59 pubmed publisher
  33. Kouwenhoven W, Veenvliet J, van Hooft J, van der Heide L, Smidt M. Engrailed 1 shapes the dopaminergic and serotonergic landscape through proper isthmic organizer maintenance and function. Biol Open. 2016;5:279-88 pubmed publisher
    ..Our work suggests a newly-discovered role for En1: the repression of Otx2, Wnt1 and canonical Wnt-signaling in R1. Overall, our results suggest that En1 is essential for proper IsO maintenance and function. ..
  34. Rouzaud Laborde C, Hanoun N, Baysal I, Rech J, Mias C, Calise D, et al. Role of endothelial AADC in cardiac synthesis of serotonin and nitrates accumulation. PLoS ONE. 2012;7:e34893 pubmed publisher
    ..and localization of 5-HT synthesizing enzyme tryptophan hydroxylase (TPH) and L-aromatic amino acid decarboxylase (AADC) in the heart. We also evaluated their involvement in cardiac production of 5-HT...
  35. Hermanson E, Joseph B, Castro D, Lindqvist E, Aarnisalo P, Wallén A, et al. Nurr1 regulates dopamine synthesis and storage in MN9D dopamine cells. Exp Cell Res. 2003;288:324-34 pubmed
    ..that Nurr1, but not retinoids, increases DA content and the expression of aromatic L-amino acid decarboxylase (AADC) and vesicular monoamine transporter-2 (VMAT2) in MN9D cells...
  36. Lengeling A, Zimmer W, Goodman S, Ma Y, Bloom M, Bruneau G, et al. Exclusion of two candidate genes, Spnb-2 and Dcd, for the wobbler spinal muscular atrophy gene on proximal mouse chromosome 11. Mamm Genome. 1994;5:163-6 pubmed
  37. Caine C, Shohat M, Kim J, Nakanishi K, Homma S, Mosharov E, et al. A pathogenic S250F missense mutation results in a mouse model of mild aromatic l-amino acid decarboxylase (AADC) deficiency. Hum Mol Genet. 2017;26:4406-4415 pubmed publisher
    Homozygous mutations in the aromatic l-amino acid decarboxylase (AADC) gene result in a severe depletion of its namesake protein, triggering a debilitating and often fatal form of infantile Parkinsonism known as AADC deficiency...
  38. Bonnin A, Goeden N, Chen K, Wilson M, King J, Shih J, et al. A transient placental source of serotonin for the fetal forebrain. Nature. 2011;472:347-50 pubmed publisher
  39. Duchemin A, Neff N, Hadjiconstantinou M. Aromatic L-amino acid decarboxylase phosphorylation and activation by PKGIalpha in vitro. J Neurochem. 2010;114:542-52 pubmed publisher
    ..Neuronal AAAD is best known for its role in the biosynthesis of catecholamines, indoleamines and trace amines in the nervous system, and the biological importance of PKGIalpha phosphorylation in these processes remains to be determined. ..
  40. Nouri N, Awatramani R. A novel floor plate boundary defined by adjacent En1 and Dbx1 microdomains distinguishes midbrain dopamine and hypothalamic neurons. Development. 2017;144:916-927 pubmed publisher
    ..Our findings provide new insights into subdivisions in the mdFP, and will impact current strategies for the conversion of stem cells into DA neurons. ..
  41. López Contreras A, Galindo J, Lopez Garcia C, Castells M, Cremades A, Peñafiel R. Opposite sexual dimorphism of 3,4-dihydroxyphenylalanine decarboxylase in the kidney and small intestine of mice. J Endocrinol. 2008;196:615-24 pubmed publisher
    3,4-Dihydroxyphenylalanine decarboxylase (DDC; also known as l-amino acid decarboxylase) is involved in the synthesis of dopamine, norepinephrine, and serotonin, and also acts as an androgen receptor co-regulator protein...