DDC

Chr 7AR

dopa decarboxylase

Also known as: AADC

The encoded protein catalyzes the decarboxylation of L-3,4-dihydroxyphenylalanine (DOPA) to dopamine, L-5-hydroxytryptophan to serotonin and L-tryptophan to tryptamine. Defects in this gene are the cause of aromatic L-amino-acid decarboxylase deficiency (AADCD). AADCD deficiency is an inborn error in neurotransmitter metabolism that leads to combined serotonin and catecholamine deficiency. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2011]

Primary Disease Associations & Inheritance

Aromatic L-amino acid decarboxylase deficiencyMIM #608643
AR
UniProtAromatic L-amino-acid decarboxylase deficiency
0
ClinVar variants
0
Pathogenic / LP
0.00
pLI score
12
Active trials
Clinical SummaryDDC
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

clinvarCount: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?LOEUF (Loss-of-function Observed/Expected Upper bound Fraction) is the upper bound of the 90% CI for LoF OE — the preferred gnomAD v4 metric. Lower = more intolerant to LoF. LOEUF < 0.35 = highly constrained.
0.92LOEUF
pLI 0.000
Z-score 1.90
OE 0.60 (0.410.92)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?Missense Z-score: standard deviations fewer missense variants observed vs. expected. Z > 3.09 (p < 0.001) = gene does not tolerate missense variation. OE missense < 0.6 is also considered constrained.
0.41Z-score
OE missense 0.93 (0.841.03)
258 obs / 277.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?Shaded band = 90% confidence interval. Vertical tick = point estimate. Grey threshold line = gnomAD constraint cutoff for that variant class.
LoF OE?Ratio of observed to expected LoF variants. Upper CI bound (LOEUF) ≤ 0.35 = strong LoF constraint signal.0.60 (0.410.92)
00.351.4
Missense OE?Ratio of observed to expected missense variants. OE ≤ 0.6 = fewer missense variants than expected by chance.0.93 (0.841.03)
00.61.4
Synonymous OE?Control metric — synonymous variants are largely neutral and expected near OE = 1.0. Significant deviation may indicate annotation issues.1.09
01.21.6
LoF obs/exp: 16 / 26.5Missense obs/exp: 258 / 277.2Syn Z: -0.72

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DDC · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Gene2Phenotype Curations

DDC-related aromatic L-amino acid decarboxylase deficiency

definitive
ARLoss Of FunctionAbsent Gene Product
Dev. Disorders
G2P ↗

Gene2Phenotype curations · DECIPHER consortium patient cohort (public variants) · deciphergenomics.org

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

DOPA DECARBOXYLASE; DDC
MIM #107930 · *

Aromatic L-amino acid decarboxylase deficiency

MIM #608643

Molecular basis of disorder known

Autosomal recessive
Clinical Literature
Landmark / reviewRecent case evidence

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Breast AdenocarcinomaHormone Receptor PositiveStage IA Breast Cancer AJCC v7

Hormone Therapy With or Without Combination Chemotherapy in Treating Women Who Have Undergone Surgery for Node-Negative Breast Cancer (The TAILORx Trial)

ACTIVE NOT RECRUITING
NCT00310180Phase PHASE3National Cancer Institute (NCI)Started 2006-04-07
AnastrozoleExemestaneLaboratory Biomarker Analysis
Acinar Cell CarcinomaAdenoid Cystic CarcinomaAdrenal Cortical Carcinoma

Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

ACTIVE NOT RECRUITING
NCT02834013Phase PHASE2National Cancer Institute (NCI)Started 2017-01-30
Biospecimen CollectionComputed TomographyEchocardiography Test
Intracranial MeningiomaRecurrent MeningiomaNF2 Gene Mutation

Vismodegib, FAK Inhibitor GSK2256098, Capivasertib, and Abemaciclib in Treating Patients With Progressive Meningiomas

RECRUITING
NCT02523014Phase PHASE2Alliance for Clinical Trials in OncologyStarted 2015-09-28
VismodegibFAK Inhibitor GSK2256098Capivasertib
Breast CancerCognitive Impairments

Cognitive Training for Cancer Related Cognitive Impairment in Breast Cancer Survivors

RECRUITING
NCT05896189Phase NANRG OncologyStarted 2024-04-12
Arm 1: Computerized Cognitive Training-Global Stimulation GamesArm 2: Computerized Cognitive Training-Neuroplasticity Games
Recurrent Non-Small Cell Lung CarcinomaStage IV Non-Small Cell Lung Cancer

S1403, Afatinib Dimaleate With or Without Cetuximab in Treating Patients With Newly Diagnosed Stage IV or Recurrent, EGFR Mutation Positive Non-small Cell Lung Cancer

ACTIVE NOT RECRUITING
NCT02438722Phase PHASE2, PHASE3SWOG Cancer Research NetworkStarted 2015-05-07
Afatinib DimaleateCetuximabLaboratory Biomarker Analysis
Colorectal Neuroendocrine Tumor G1Gastric Neuroendocrine Tumor G1Neuroendocrine Neoplasm

Octreotide Acetate and Recombinant Interferon Alfa-2b or Bevacizumab in Treating Patients With Metastatic or Locally Advanced, High-Risk Neuroendocrine Tumor

ACTIVE NOT RECRUITING
NCT00569127Phase PHASE3National Cancer Institute (NCI)Started 2007-12-01
BevacizumabLaboratory Biomarker AnalysisOctreotide Acetate
Colon AdenocarcinomaStage IIA Colon Cancer AJCC v7Stage IIB Colon Cancer AJCC v7

Oxaliplatin, Leucovorin, and Fluorouracil With or Without Bevacizumab in Treating Patients Who Have Undergone Surgery for Stage II Colon Cancer

ACTIVE NOT RECRUITING
NCT00217737Phase PHASE3National Cancer Institute (NCI)Started 2005-09-06
BevacizumabFluorouracilLeucovorin
Aromatic L-amino Acid Decarboxylase Deficiency

A Trial to Evaluate Safety and Efficacy of a Product Named VGN-R09b in Severe AADC Deficiency

NOT YET RECRUITING
NCT06432140Phase PHASE1Shanghai Vitalgen BioPharma Co., Ltd.Started 2024-05
VGN-R09b injection
Plasma Cell Myeloma

Bortezomib or Carfilzomib With Lenalidomide and Dexamethasone in Treating Patients With Newly Diagnosed Multiple Myeloma

ACTIVE NOT RECRUITING
NCT01863550Phase PHASE3ECOG-ACRIN Cancer Research GroupStarted 2013-12-06
BortezomibCarfilzomibDexamethasone
Recurrent Diffuse Large B-Cell Lymphoma Activated B-Cell TypeRefractory Diffuse Large B-Cell Lymphoma Activated B-Cell Type

Ibrutinib Before and After Stem Cell Transplant in Treating Patients With Relapsed or Refractory Diffuse Large B-cell Lymphoma

ACTIVE NOT RECRUITING
NCT02443077Phase PHASE3National Cancer Institute (NCI)Started 2016-10-12
Autologous Bone Marrow TransplantationAutologous Hematopoietic Stem Cell TransplantationCarmustine
OsteosarcomaSoft Tissue Sarcoma

Safety and Efficacy of DIT309 in Advanced Bone and Soft Tissue Sarcomas

NOT YET RECRUITING
NCT07052383Phase PHASE1Tcelltech Inc.Started 2025-08-20
DIT309 cell injection
KRAS Gene MutationRecurrent Lung Non-Small Cell CarcinomaStage IV Lung Non-Small Cell Cancer AJCC v7

Trametinib and Docetaxel in Treating Patients With Recurrent or Stage IV KRAS Mutation Positive Non-small Cell Lung Cancer

ACTIVE NOT RECRUITING
NCT02642042Phase PHASE2National Cancer Institute (NCI)Started 2016-07-18
DocetaxelLaboratory Biomarker AnalysisTrametinib