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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ACTN3
actinin alpha 3
Chromosome 11 · 11q13.2
NCBI Gene: 89Ensembl: ENSG00000248746.6HGNC: HGNC:165UniProt: A0A087WSZ2
268PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingfocal adhesionidentical protein bindingtransmembrane transporter bindingAbnormality of the skeletal systemmental or behavioural disordertotal hip arthroplastyosteoarthritis, hip
✦AI Summary

ACTN3 encodes α-actinin-3, an F-actin cross-linking protein localized to the Z-disc of skeletal muscle that bundles actin filaments and anchors them to intracellular structures 1. Expression is restricted to type 2 (fast-twitch) fibers responsible for forceful, high-velocity contractions 1. A common R577X stop-codon polymorphism results in α-actinin-3 deficiency in approximately 18% of Caucasians 2, which does not cause disease but is compensated by the homologous protein α-actinin-2 2. The RR genotype associates with enhanced sprint and power performance, with elite sprint athletes showing significantly higher R allele frequencies 2. Conversely, the XX genotype may confer advantages in endurance activities through a metabolic shift toward oxidative metabolism and increased fatigue resistance 1. In aging populations, ACTN3 genotype influences muscle phenotype and bone mineral density 3, though associations with sarcopenia require further investigation 4. A recent missense variant increases filament flexibility, reducing maximal force in type IIA fibers 5. The ACTN3 R577X polymorphism also associates with joint range of motion, with XX and RX genotypes showing greater flexibility than RR 6. Gene dosage studies demonstrate that ACTN3 overexpression is detrimental to muscle function, highlighting the sensitive balance of sarcomeric protein stoichiometry 7.

Sources cited
1
ACTN3 R577X polymorphism association with elite sprint athletic performance and the RR genotype advantage
PMID: 12879365
2
ACTN3 expression restricted to type 2 fibers, Z-disc localization, and metabolic shift in XX genotype toward oxidative metabolism
PMID: 19696509
3
ACTN3 genotype impact on muscle phenotype, bone mineral density, and metabolic disorders in elderly populations
PMID: 29416549
4
Investigation of ACTN3 R577X polymorphism relationship with sarcopenia in older adults
PMID: 34677800
5
ACTN3 missense variant effects on filament flexibility and maximal force in type IIA muscle fibers
PMID: 36071172
6
ACTN3 R577X polymorphism association with range of motion, showing XX/RX genotypes have greater flexibility than RR
PMID: 36787803
7
ACTN3 gene dosage effects showing overexpression is detrimental to muscle function and primary role in muscle metabolism
PMID: 29706347
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.51Moderate
mental or behavioural disorderOpen Targets
0.33Weak
osteoarthritis, hipOpen Targets
0.32Weak
total hip arthroplastyOpen Targets
0.32Weak
ACTN3 deficiencyOpen Targets
0.18Weak
asthmaOpen Targets
0.12Weak
ulcerative colitisOpen Targets
0.08Suggestive
bipolar disorderOpen Targets
0.07Suggestive
Abruptio PlacentaeOpen Targets
0.06Suggestive
insomniaOpen Targets
0.05Suggestive
Duchenne muscular dystrophyOpen Targets
0.05Suggestive
sarcopeniaOpen Targets
0.05Suggestive
altitude sicknessOpen Targets
0.03Suggestive
acute myeloid leukemiaOpen Targets
0.02Suggestive
type 2 diabetes mellitusOpen Targets
0.02Suggestive
muscular dystrophyOpen Targets
0.02Suggestive
congenital muscular dystrophyOpen Targets
0.02Suggestive
chronic hepatitis B virus infectionOpen Targets
0.01Suggestive
obesityOpen Targets
0.01Suggestive
bruxismOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MYH2Protein interaction99%MYL1Protein interaction99%MYOZ1Protein interaction99%MYH7Protein interaction96%MYL2Protein interaction95%TCAPProtein interaction95%
Tissue Expression6 tissues
Ovary
100%
Lung
67%
Brain
43%
Liver
26%
Heart
15%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
ACTN3MYH2MYL1MYOZ1MYH7MYL2TCAP
PROTEIN STRUCTURE
Preparing viewer…
PDB1WKU · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.94LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.79 [0.67–0.94]
RankingsWhere ACTN3 stands among ~20K protein-coding genes
  • #1,380of 20,598
    Most Researched268 · top 10%
  • #8,795of 17,882
    Most Constrained (LOEUF)0.94
Genes detectedACTN3
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
ACTN3 genotype is associated with human elite athletic performance.
PMID: 12879365
Am J Hum Genet · 2003
1.00
2
Genome-wide association analyses of physical activity and sedentary behavior provide insights into underlying mechanisms and roles in disease prevention.
PMID: 36071172
Nat Genet · 2022
0.90
3
The Role of
PMID: 41751596
Genes (Basel) · 2026
0.88
4
The Association between ACTN3 R577X Polymorphism and Range of Motion: A Systematic Review and Meta-analysis.
PMID: 36787803
Int J Sports Med · 2023
0.80
5
Genetic and Epigenetic Modulation of Cell Functions by Physical Exercise.
PMID: 31888150
Genes (Basel) · 2019
0.72