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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CACNA1D
calcium voltage-gated channel subunit alpha1 D
Chromosome 3 · 3p21.1
NCBI Gene: 776Ensembl: ENSG00000157388.20HGNC: HGNC:1391UniProt: A0A1B0GUN6
110PubMed Papers
22Diseases
41Drugs
21Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedHub GeneIon ChannelTransporter
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
calcium ion transportcalcium ion importsensory perception of soundcalcium channel activityaldosterone-producing adenoma with seizures and neurological abnormalitieshypertensionsinoatrial node dysfunction and deafnesscardiovascular disease
✦AI Summary

CACNA1D encodes the pore-forming α1 subunit of Cav1.3 L-type voltage-gated calcium channels, which mediate calcium ion entry into excitable cells and regulate calcium-dependent processes including muscle contraction, hormone/neurotransmitter release, and gene expression 1. The channel is particularly important in cardiac conduction, sensory perception, and neuronal function [GO annotations provided]. CACNA1D variants have been associated with multiple disease phenotypes. Loss-of-function mutations cause deafness and bradycardia through reduced Cav1.3 activity 1, while gain-of-function de novo variants are linked to neurodevelopmental disorders including autism spectrum disorder and intellectual disability 213. CACNA1D mutations also contribute to congenital hyperinsulinism, causing unregulated insulin secretion and neonatal hypoglycemia 4. Additionally, CACNA1D variants have been identified in familial advanced sleep phase disorder, with altered channel dynamics affecting circadian light entrainment 5. The A749G variant in mice reproduces developmental delay, social deficits, and hyperactivity through altered dopamine neuron excitability 3. Disease-associated variants demonstrate altered channel gating and kinetics, and L-type calcium channel inhibitors like isradipine represent potential therapeutic approaches 1. CACNA1D also serves as a subtype-specific therapeutic target in pancreatic neuroendocrine tumors 6.

Sources cited
1
CACNA1D encodes Cav1.3 α1 subunit; loss-of-function causes deafness/bradycardia; gain-of-function variants linked to hypertension, congenital hypoglycemia, autism; dihydropyridines as potential treatment
PMID: 36592224
2
CACNA1D mutations associated with intellectual disability/global developmental delay; both gain- and loss-of-function variants implicated; gain-of-function shows more severe phenotype
PMID: 33985586
3
CACNA1D mutations cause congenital hyperinsulinism through dysregulation of insulin secretion from pancreatic β-cells
PMID: 34547194
4
CACNA1D variants identified in familial advanced sleep phase with altered channel dynamics affecting light entrainment
PMID: 40460120
5
A749G CACNA1D variant induces neurodevelopmental phenotype in mice including developmental delay, social deficit, hyperactivity through altered dopamine neuron excitability
PMID: 37698939
6
CACNA1D identified as subtype-specific therapeutic target in pancreatic neuroendocrine tumors
PMID: 40185092
Disease Associationsⓘ22
aldosterone-producing adenoma with seizures and neurological abnormalitiesOpen Targets
0.76Strong
hypertensionOpen Targets
0.74Strong
sinoatrial node dysfunction and deafnessOpen Targets
0.74Strong
cardiovascular diseaseOpen Targets
0.70Moderate
atrial fibrillationOpen Targets
0.67Moderate
heart failureOpen Targets
0.67Moderate
diabetes mellitusOpen Targets
0.66Moderate
essential hypertensionOpen Targets
0.61Moderate
epilepsyOpen Targets
0.61Moderate
coronary artery diseaseOpen Targets
0.61Moderate
Prinzmetal's anginaOpen Targets
0.60Moderate
SeizureOpen Targets
0.60Moderate
fibromyalgiaOpen Targets
0.60Moderate
neuropathic painOpen Targets
0.60Moderate
angina pectorisOpen Targets
0.60Moderate
myocardial infarctionOpen Targets
0.59Moderate
restless legs syndromeOpen Targets
0.58Moderate
neuralgiaOpen Targets
0.58Moderate
hyperlipidemiaOpen Targets
0.57Moderate
anxiety disorderOpen Targets
0.57Moderate
Primary aldosteronism, seizures, and neurologic abnormalitiesUniProt
Sinoatrial node dysfunction and deafnessUniProt
Pathogenic Variants21
NM_000720.4(CACNA1D):c.1208_1209insGGG (p.Gly403dup)Pathogenic
Sinoatrial node dysfunction and deafness|not provided
★★☆☆2024→ Residue 403
NM_000720.4(CACNA1D):c.1208G>A (p.Gly403Asp)Pathogenic
Aldosterone-producing adenoma with seizures and neurological abnormalities|not provided
★★☆☆2024→ Residue 403
NM_001128840.3(CACNA1D):c.1955C>T (p.Ser652Leu)Pathogenic
See cases|not provided
★★☆☆2024→ Residue 652
NM_001128840.3(CACNA1D):c.2969G>A (p.Arg990His)Likely pathogenic
not provided
★☆☆☆2025→ Residue 990
NM_001128840.3(CACNA1D):c.3323A>G (p.Tyr1108Cys)Likely pathogenic
not provided
★☆☆☆2025→ Residue 1108
NM_001128840.3(CACNA1D):c.2241C>G (p.Phe747Leu)Likely pathogenic
Aldosterone-producing adenoma with seizures and neurological abnormalities
★☆☆☆2025→ Residue 747
NM_001128840.3(CACNA1D):c.1634A>G (p.Tyr545Cys)Likely pathogenic
not provided
★☆☆☆2023→ Residue 545
NM_001128840.3(CACNA1D):c.2249T>G (p.Ile750Ser)Likely pathogenic
not provided
★☆☆☆2023→ Residue 750
NM_001128840.3(CACNA1D):c.2907del (p.Phe970fs)Likely pathogenic
Sinoatrial node dysfunction and deafness
★☆☆☆2023→ Residue 970
NM_001128840.3(CACNA1D):c.1856G>C (p.Arg619Pro)Pathogenic
not provided
★☆☆☆2023→ Residue 619
NM_001128840.3(CACNA1D):c.1201G>C (p.Val401Leu)Likely pathogenic
See cases
★☆☆☆2022→ Residue 401
NM_001128840.3(CACNA1D):c.776T>A (p.Val259Asp)Pathogenic
not provided
★☆☆☆2021→ Residue 259
NM_001128840.3(CACNA1D):c.3964C>T (p.Arg1322Ter)Pathogenic
Inborn genetic diseases
★☆☆☆2021→ Residue 1322
NM_001128840.3(CACNA1D):c.2248A>T (p.Ile750Phe)Likely pathogenic
Sinoatrial node dysfunction and deafness
★☆☆☆2019→ Residue 750
NM_001128840.3(CACNA1D):c.2245G>A (p.Ala749Thr)Likely pathogenic
not provided
★☆☆☆2018→ Residue 749
NM_001128840.3(CACNA1D):c.4362+1G>CLikely pathogenic
Inborn genetic diseases
★☆☆☆2017
NM_001128840.3(CACNA1D):c.698G>A (p.Gly233Asp)Likely pathogenic
Sinoatrial node dysfunction and deafness;Aldosterone-producing adenoma with seizures and neurological abnormalities
★☆☆☆→ Residue 233
NM_001128840.3(CACNA1D):c.2239T>C (p.Phe747Leu)Pathogenic
Congenital disorder of glycosylation, type Iw, autosomal dominant
☆☆☆☆2023→ Residue 747
NM_001128840.3(CACNA1D):c.2222-1G>APathogenic
Aldosterone-producing adenoma with seizures and neurological abnormalities
☆☆☆☆2022
NM_001128840.3(CACNA1D):c.2250C>G (p.Ile750Met)Pathogenic
Aldosterone-producing adenoma with seizures and neurological abnormalities|not provided
☆☆☆☆2013→ Residue 750
View on ClinVar ↗
Drug Targets41
AMLODIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
AMLODIPINE BENZOATEApproved
Voltage-gated L-type calcium channel blocker
hypertension
AMLODIPINE BESYLATEApproved
Voltage-gated L-type calcium channel blocker
Prinzmetal's angina
ATAGABALINPhase II
Voltage-gated calcium channel modulator
insomnia
AZD1305Phase II
HERG blocker
atrial flutter
AZELNIDIPINEApproved
Voltage-gated L-type calcium channel blocker
hypertension
BARNIDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
BENIDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
BEPRIDILApproved
Voltage-gated calcium channel blocker
cardiovascular disease
CILNIDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
CINNARIZINEApproved
Histamine H1 receptor antagonist
CLEVIDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
DILTIAZEMApproved
Voltage-gated L-type calcium channel blocker
hemorrhoid
DILTIAZEM HYDROCHLORIDEApproved
Voltage-gated L-type calcium channel blocker
angina pectoris
DRONEDARONEApproved
Sodium channel alpha subunit blocker
cardiac arrhythmia
DRONEDARONE HYDROCHLORIDEApproved
Sodium channel alpha subunit blocker
atrial fibrillation
ELPETRIGINEPhase I
Sodium channel protein type II alpha subunit blocker
bipolar disorder
FELODIPINEApproved
Mineralocorticoid receptor antagonist
hypertension
GABAPENTINApproved
Voltage-gated calcium channel modulator
epilepsy
GABAPENTIN ENACARBILApproved
Voltage-gated calcium channel modulator
restless legs syndrome
IMAGABALINPhase III
Voltage-gated calcium channel modulator
generalized anxiety disorder
ISRADIPINEApproved
Voltage-gated L-type calcium channel blocker
hypertension
LACIDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
LERCANIDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
LEVAMLODIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
LEVAMLODIPINE MALEATEApproved
Voltage-gated L-type calcium channel blocker
hypertension
MANIDIPINE 6300Approved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
NICARDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
NICARDIPINE HYDROCHLORIDEApproved
Voltage-gated L-type calcium channel blocker
hypertension
NIFEDIPINEApproved
Voltage-gated L-type calcium channel blocker
hypertension
NILVADIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
NIMODIPINEApproved
Mineralocorticoid receptor antagonist
NISOLDIPINEApproved
Voltage-gated L-type calcium channel blocker
hypertension
NITRENDIPINEApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
PHLOROGLUCINOLApproved
Voltage-gated calcium channel blocker
gastrointestinal disease
PREGABALINApproved
Voltage-gated calcium channel modulator
neuropathic pain
SULOCTIDILApproved
Voltage-gated calcium channel blocker
cardiovascular disease
TERODILINEApproved
Muscarinic acetylcholine receptor antagonist
Urinary incontinence
TERODILINE HYDROCHLORIDEUNKNOWN
Muscarinic acetylcholine receptor antagonist
VERAPAMILApproved
Voltage-gated L-type calcium channel blocker
cardiovascular disease
VERAPAMIL HYDROCHLORIDEApproved
Voltage-gated L-type calcium channel blocker
hypertension
Related Genes
CALML3Protein interaction100%CALML6Protein interaction100%CALML5Protein interaction100%CALM3Protein interaction100%CALML4Protein interaction100%CACHD1Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
84%
Liver
50%
Brain
32%
Ovary
12%
Heart
4%
Gene Interaction Network
Click a node to explore
CACNA1DCALML3CALML6CALML5CALM3CALML4CACHD1
PROTEIN STRUCTURE
Preparing viewer…
PDB3LV3 · 1.94 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.25Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.19 [0.15–0.25]
RankingsWhere CACNA1D stands among ~20K protein-coding genes
  • #4,308of 20,598
    Most Researched110 · top quartile
  • #50of 1,025
    FDA-Approved Drug Targets36 · top 5%
  • #2,140of 5,498
    Most Pathogenic Variants21
  • #786of 17,882
    Most Constrained (LOEUF)0.25 · top 5%
Genes detectedCACNA1D
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Autophagy in the physiological endometrium and cancer.
PMID: 32401642
Autophagy · 2021
1.00
2
Calcium channelopathies and intellectual disability: a systematic review.
PMID: 33985586
Orphanet J Rare Dis · 2021
0.90
3
Human Genetics of Ventricular Septal Defect.
PMID: 38884729
Adv Exp Med Biol · 2024
0.80
4
Proteogenomic characterization of non-functional pancreatic neuroendocrine tumors unravels clinically relevant subgroups.
PMID: 40185092
Cancer Cell · 2025
0.70
5
Congenital hyperinsulinism: recent updates on molecular mechanisms, diagnosis and management.
PMID: 34547194
J Pediatr Endocrinol Metab · 2022
0.60