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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
PRRT2
proline rich transmembrane protein 2
Chromosome 16 Β· 16p11.2
NCBI Gene: 112476Ensembl: ENSG00000167371.22HGNC: HGNC:30500UniProt: Q7Z6L0
144PubMed Papers
23Diseases
0Drugs
158Pathogenic Variants
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
plasma membraneneuromuscular process controlling postureprotein bindingaxon terminusepisodic kinesigenic dyskinesia 1infantile convulsions and choreoathetosisbenign familial infantile epilepsyepisodic kinesigenic dyskinesia
✦AI Summary

PRRT2 (proline rich transmembrane protein 2) is a presynaptic membrane protein that plays a critical role in neurotransmitter release and synaptic transmission regulation 1. As a component involved in synaptic vesicle function, PRRT2 regulates calcium-sensing in presynaptic terminals and modulates SNARE complex formation, thereby controlling short-term synaptic facilitation 1. The protein is essential for proper neuronal communication, particularly in the control of movement and seizure susceptibility. PRRT2 mutations are the leading genetic cause of paroxysmal kinesigenic dyskinesia (PKD), accounting for the majority of cases worldwide with autosomal dominant inheritance and incomplete penetrance 2. PKD is characterized by recurrent, transient episodes of involuntary movements triggered by sudden voluntary actions 32. PRRT2 is also associated with benign familial infantile seizures and represents the most common single-gene epilepsy, with an estimated incidence of 1 per 9,970 live births 1. Notably, 85.7% of PRRT2 variants are inherited rather than de novo 4. The high frequency of PRRT2 mutations in genetic testing panels highlights its clinical significance, making it a priority target for molecular diagnostic testing in patients with early-onset epilepsy and movement disorders 41.

Sources cited
1
PRRT2 is the most common single-gene epilepsy with incidence of 1 per 9,970 live births and functions in synaptic transmission
PMID: 31302675
2
PRRT2 is the first identified and major causative gene of paroxysmal kinesigenic dyskinesia with autosomal dominant inheritance
PMID: 33588936
3
PRRT2 is involved in paroxysmal kinesigenic dyskinesia characterized by movement episodes triggered by sudden voluntary actions
PMID: 29735119
4
85.7% of PRRT2 variants are inherited and it's among high-yield genes for epilepsy genetic testing
PMID: 29655203
⚠Limited data available β€” This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜23
episodic kinesigenic dyskinesia 1Open Targets
0.82Strong
infantile convulsions and choreoathetosisOpen Targets
0.81Strong
benign familial infantile epilepsyOpen Targets
0.78Strong
episodic kinesigenic dyskinesiaOpen Targets
0.73Strong
genetic disorderOpen Targets
0.53Moderate
SeizureOpen Targets
0.52Moderate
PRRT2-associated paroxysmal movement disorderOpen Targets
0.49Moderate
neurodegenerative diseaseOpen Targets
0.47Moderate
autosomal recessive non-syndromic intellectual disabilityOpen Targets
0.42Moderate
Paroxysmal exertion-induced dyskinesiaOpen Targets
0.38Weak
paroxysmal nonkinesigenic dyskinesiaOpen Targets
0.38Weak
Generalized epilepsy with febrile seizures-plusOpen Targets
0.38Weak
generalized epilepsy with febrile seizures plusOpen Targets
0.38Weak
familial or sporadic hemiplegic migraineOpen Targets
0.37Weak
paroxysmal nonkinesigenic dyskinesia 1Open Targets
0.35Weak
Neurodevelopmental delayOpen Targets
0.35Weak
self-limited familial infantile epilepsyOpen Targets
0.34Weak
Benign familial neonatal-infantile seizuresOpen Targets
0.34Weak
movement disorderOpen Targets
0.20Weak
epilepsyOpen Targets
0.20Weak
Convulsions, familial infantile, with paroxysmal choreoathetosisUniProt
Episodic kinesigenic dyskinesia 1UniProt
Seizures, benign familial infantile, 2UniProt
Pathogenic Variants158
NM_145239.3(PRRT2):c.649dup (p.Arg217fs)Pathogenic
Episodic kinesigenic dyskinesia 1|Infantile convulsions and choreoathetosis|not provided|Seizure|Seizures, benign familial infantile, 2|Episodic kinesigenic dyskinesia|Seizures, benign familial infantile, 2;Episodic kinesigenic dyskinesia 1;Infantile convulsions and choreoathetosis|Paroxysmal nonkinesigenic dyskinesia 1|PRRT2 insufficiency|PRRT2-Associated Paroxysmal Movement Disorders|Neurodevelopmental delay|PRRT2-Related Disorder|Inborn genetic diseases|Self-limited familial infantile epilepsy|See cases|Convulsions|Seizures, benign familial infantile, 2;Episodic kinesigenic dyskinesia 1|Paroxysmal central nervous system disorders|Autosomal dominant PRRT2-related disorders
β˜…β˜…β˜†β˜†2026β†’ Residue 217
NM_145239.3(PRRT2):c.649del (p.Arg217fs)Pathogenic
Episodic kinesigenic dyskinesia 1|not provided|Episodic kinesigenic dyskinesia|Seizures, benign familial infantile, 2|Seizures, benign familial infantile, 2;Episodic kinesigenic dyskinesia 1;Infantile convulsions and choreoathetosis|Inborn genetic diseases|Infantile convulsions and choreoathetosis|PRRT2-Related Disorder|PRRT2-associated paroxysmal movement disorder|Benign familial neonatal-infantile seizures 1
β˜…β˜…β˜†β˜†2026β†’ Residue 217
NM_145239.3(PRRT2):c.880-34G>APathogenic
not provided|Episodic kinesigenic dyskinesia|Seizures, benign familial infantile, 2|Episodic kinesigenic dyskinesia 1;Infantile convulsions and choreoathetosis|Inborn genetic diseases
β˜…β˜…β˜†β˜†2025
NM_145239.3(PRRT2):c.210_211del (p.Glu70fs)Pathogenic
not provided|Episodic kinesigenic dyskinesia
β˜…β˜…β˜†β˜†2025β†’ Residue 70
NM_145239.3(PRRT2):c.679C>T (p.Arg227Ter)Pathogenic
Episodic kinesigenic dyskinesia|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 227
NM_145239.3(PRRT2):c.629dup (p.Ala211fs)Pathogenic
Infantile convulsions and choreoathetosis|not provided|Episodic kinesigenic dyskinesia 1|Episodic kinesigenic dyskinesia|PRRT2-associated paroxysmal movement disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 211
NM_145239.3(PRRT2):c.604_607del (p.Ser202fs)Pathogenic
not provided|Episodic kinesigenic dyskinesia|Inborn genetic diseases
β˜…β˜…β˜†β˜†2025β†’ Residue 202
NM_145239.3(PRRT2):c.898C>T (p.Gln300Ter)Pathogenic
not provided|Episodic kinesigenic dyskinesia
β˜…β˜…β˜†β˜†2025β†’ Residue 300
NM_145239.3(PRRT2):c.107_110del (p.Gln36fs)Pathogenic
Seizures, benign familial infantile, 2;Episodic kinesigenic dyskinesia 1;Infantile convulsions and choreoathetosis|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 36
NM_145239.3(PRRT2):c.514_517del (p.Ser172fs)Pathogenic
Episodic kinesigenic dyskinesia 1|Episodic kinesigenic dyskinesia|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 172
NM_145239.3(PRRT2):c.895C>T (p.Gln299Ter)Pathogenic
Episodic kinesigenic dyskinesia|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 299
NM_145239.3(PRRT2):c.650del (p.Arg217fs)Pathogenic
Seizures, benign familial infantile, 2|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 217
NM_145239.3(PRRT2):c.916_934del (p.Gly305_Ala306insTer)Pathogenic
Episodic kinesigenic dyskinesia|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 305
NM_145239.3(PRRT2):c.516dup (p.Glu173Ter)Pathogenic
Infantile convulsions and choreoathetosis|Episodic kinesigenic dyskinesia|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 173
NM_145239.3(PRRT2):c.219_220del (p.Glu73fs)Pathogenic
not provided|Episodic kinesigenic dyskinesia
β˜…β˜…β˜†β˜†2024β†’ Residue 73
NM_145239.3(PRRT2):c.921del (p.Gln307fs)Pathogenic
Episodic kinesigenic dyskinesia|Inborn genetic diseases
β˜…β˜…β˜†β˜†2024β†’ Residue 307
NM_145239.3(PRRT2):c.649C>T (p.Arg217Ter)Pathogenic
Episodic kinesigenic dyskinesia|not provided|Infantile convulsions and choreoathetosis|Seizures, benign familial infantile, 2|PRRT2-associated paroxysmal movement disorder
β˜…β˜…β˜†β˜†2024β†’ Residue 217
NM_145239.3(PRRT2):c.971G>A (p.Gly324Glu)Pathogenic
Episodic kinesigenic dyskinesia|not provided|Seizure
β˜…β˜…β˜†β˜†2024β†’ Residue 324
NM_145239.3(PRRT2):c.879+1G>TPathogenic
Episodic kinesigenic dyskinesia|PRRT2-Related Disorder
β˜…β˜…β˜†β˜†2024
NM_145239.3(PRRT2):c.971G>T (p.Gly324Val)Likely pathogenic
Episodic kinesigenic dyskinesia|Episodic kinesigenic dyskinesia and familial infantile convulsions with paroxysmal choreoathetosis
β˜…β˜…β˜†β˜†2024β†’ Residue 324
View on ClinVar β†—
Related Genes
SYT1Protein interaction98%PNKDProtein interaction96%CACNA1AProtein interaction88%ATP1A2Protein interaction74%KCNQ2Protein interaction74%SCN1AProtein interaction74%
Tissue Expression6 tissues
Ovary
100%
Brain
32%
Lung
5%
Heart
4%
Liver
2%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
PRRT2SYT1PNKDCACNA1AATP1A2KCNQ2SCN1A
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q7Z6L0
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.63LoF Tolerant
pLIβ“˜
0.66Intermediate
Observed/Expected LoF0.36 [0.22–0.63]
RankingsWhere PRRT2 stands among ~20K protein-coding genes
  • #3,169of 20,598
    Most Researched144 Β· top quartile
  • #471of 5,498
    Most Pathogenic Variants158 Β· top 10%
  • #4,486of 17,882
    Most Constrained (LOEUF)0.63
Genes detectedPRRT2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
International consensus on diagnosis and management of Dravet syndrome.
PMID: 35490361
Epilepsia Β· 2022
1.00
2
Episodic Ataxias: Primary and Secondary Etiologies, Treatment, and Classification Approaches.
PMID: 37008993
Tremor Other Hyperkinet Mov (N Y) Β· 2023
0.90
3
Diagnostic outcomes for genetic testing of 70 genes in 8565 patients with epilepsy and neurodevelopmental disorders.
PMID: 29655203
Epilepsia Β· 2018
0.80
4
Epidemiology of Developmental and Epileptic Encephalopathy and of Intellectual Disability and Epilepsy in Children.
PMID: 36581463
Neurology Β· 2023
0.70
5
Incidence and phenotypes of childhood-onset genetic epilepsies: a prospective population-based national cohort.
PMID: 31302675
Brain Β· 2019
0.60