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]

GeneReviewsOMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.921 OMIM phenotype
Clinical SummaryDDC
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Gene-Disease Validity (ClinGen)
aromatic L-amino acid decarboxylase deficiency · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — DDC
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.92LOEUF
pLI 0.000
Z-score 1.90
OE 0.60 (0.410.92)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.41Z-score
OE missense 0.93 (0.841.03)
258 obs / 277.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.60 (0.410.92)
00.351.4
Missense OE?0.93 (0.841.03)
00.61.4
Synonymous OE?1.09
01.21.6
LoF obs/exp: 16 / 26.5Missense obs/exp: 258 / 277.2Syn Z: -0.72
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveDDC-related aromatic L-amino acid decarboxylase deficiencyLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.78top 25%
GOF
0.7029th %ile
LOF
0.2483th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

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.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Aromatic L-amino Acid Decarboxylase Deficiency

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

ACTIVE NOT RECRUITING
NCT06432140Phase PHASE2, PHASE3Shanghai Vitalgen BioPharma Co., Ltd.Started 2024-07-02
VGN-R09b 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
Anatomic Stage I Breast Cancer AJCC v8Anatomic Stage II Breast Cancer AJCC v8Anatomic Stage III Breast Cancer AJCC v8

Additional Support Program Via Text Messaging and Telephone-Based Counseling for Breast Cancer Patients Receiving Hormonal Therapy

ACTIVE NOT RECRUITING
NCT04379570Phase PHASE3Alliance for Clinical Trials in OncologyStarted 2021-02-15
Educational InterventionText Message-based Navigation InterventionMotivational Interviewing
Previously Treated Non-Small Cell Lung Cancer

Lung-MAP: A Master Screening Protocol for Previously-Treated Non-Small Cell Lung Cancer

RECRUITING
NCT03851445Phase PHASE2, PHASE3SWOG Cancer Research NetworkStarted 2019-02-06
Screening Platform
AADC Deficiency

A Study of SmartFlow Magnetic Resonance (MR) Compatible Ventricular Cannula for Administering Eladocagene Exuparvovec to Pediatric Participants

ACTIVE NOT RECRUITING
NCT04903288Phase PHASE2PTC TherapeuticsStarted 2021-05-12
Eladocagene Exuparvovec
Ductal Breast Carcinoma In Situ

MRI and Gene Expression in Diagnosing Patients With Ductal Breast Cancer In Situ

ACTIVE NOT RECRUITING
NCT02352883Phase NAECOG-ACRIN Cancer Research GroupStarted 2015-03-25
Magnetic Resonance ImagingTherapeutic Conventional SurgeryTherapeutic Surgical Procedure
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
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
Clinical Stage III Cutaneous Melanoma AJCC v8Clinical Stage IV Cutaneous Melanoma AJCC v8Metastatic Melanoma

Dabrafenib and Trametinib Followed by Ipilimumab and Nivolumab or Ipilimumab and Nivolumab Followed by Dabrafenib and Trametinib in Treating Patients With Stage III-IV BRAFV600 Melanoma

ACTIVE NOT RECRUITING
NCT02224781Phase PHASE3National Cancer Institute (NCI)Started 2015-09-08
Biospecimen CollectionComputed TomographyDabrafenib Mesylate
Mild Cognitive Impairment

A Single-center, Self-controlled, Prospective Case Series Pilot Study to Assess the Effect of Lamivudine (3TC) on Neurocognitive Impairment Biomarkers and Type-I IFN (Interferon)-Stimulated Genes in the Plasma of Patients With Mild Cognitive Impairment (MCI)

ACTIVE NOT RECRUITING
NCT06519357Phase PHASE2Fundación FLS de Lucha Contra el Sida, las Enfermedades Infecciosas y la Promoción de la Salud y la CienciaStarted 2024-10-28
Lamivudine 300 MG
Melanoma (Skin)

High-Dose Interferon Alfa in Treating Patients With Stage II or Stage III Melanoma

ACTIVE NOT RECRUITING
NCT00003641Phase PHASE3ECOG-ACRIN Cancer Research GroupStarted 1999-03-25
interferon alfa-2bobservation
Recurrent Squamous Cell Lung CarcinomaStage IV Squamous Cell Lung Carcinoma AJCC v7

Lung-MAP: Nivolumab With or Without Ipilimumab as Second-Line Therapy in Treating Patients With Recurrent Stage IV Squamous Cell Lung Cancer and No Matching Biomarkers

ACTIVE NOT RECRUITING
NCT02785952Phase PHASE3SWOG Cancer Research NetworkStarted 2015-12-29
IpilimumabLaboratory Biomarker AnalysisNivolumab