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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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MYLK2
myosin light chain kinase 2
Chromosome 20 · 20q11.21
NCBI Gene: 85366Ensembl: ENSG00000101306.12HGNC: HGNC:16243UniProt: Q9H1R3
54PubMed Papers
21Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Kinase
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingcytoplasmnucleusendoplasmic reticulumfamilial hypertrophic cardiomyopathycardiomyopathyhypertrophic cardiomyopathy 1hypertrophic cardiomyopathy
✦AI Summary

MYLK2 encodes myosin light chain kinase 2, a calcium/calmodulin-dependent serine/threonine kinase that plays a crucial role in striated muscle contraction. The protein phosphorylates specific serine residues in the N-terminus of myosin light chains, regulating muscle filament sliding and global muscle contraction in both cardiac and skeletal muscle 1. MYLK2 functions through calcium/calmodulin-dependent signaling pathways and is essential for maintaining vascular smooth muscle cell contractility and blood pressure regulation 1. The gene has been associated with familial hypertrophic cardiomyopathy, with mutations identified in affected families showing distinct cardiac phenotypes 23. Specifically, truncating mutations like p.E380X have been linked to dilated cardiomyopathy, while missense mutations such as K324E are associated with ECG abnormalities including inverted T waves 23. Additionally, MYLK2 expression levels correlate with insulin sensitivity in skeletal muscle, suggesting a role in metabolic regulation 4. Recent genome-wide association studies have also identified MYLK2 as a potential risk locus for Parkinson's disease, though its specific role in neurodegeneration requires further investigation 5.

Sources cited
1
MYLK2 regulates vascular smooth muscle contraction through calcium/calmodulin-dependent mechanisms and blood pressure control
PMID: 40795179
2
Truncating MYLK2 mutations (p.E380X) are associated with dilated cardiomyopathy in affected family members
PMID: 33455984
3
MYLK2-K324E mutation carriers show inverted ECG T waves and contribute to cardiac phenotypes in HCM families
PMID: 25825456
4
MYLK2 protein levels differ between high and low insulin sensitivity groups in skeletal muscle
PMID: 36876056
5
MYLK2 was identified as a potentially novel risk locus for Parkinson's disease in genome-wide association studies
PMID: 38155330
Disease Associationsⓘ21
familial hypertrophic cardiomyopathyOpen Targets
0.57Moderate
cardiomyopathyOpen Targets
0.34Weak
hypertrophic cardiomyopathy 1Open Targets
0.26Weak
hypertrophic cardiomyopathyOpen Targets
0.23Weak
Rare familial disorder with hypertrophic cardiomyopathyOpen Targets
0.21Weak
Parkinson diseaseOpen Targets
0.14Weak
ventricular tachycardiaOpen Targets
0.12Weak
dilated cardiomyopathy 1KKOpen Targets
0.12Weak
Left ventricular noncompaction cardiomyopathyOpen Targets
0.12Weak
Angiokeratoma corporis diffusumOpen Targets
0.11Weak
Fabry diseaseOpen Targets
0.11Weak
prostate carcinomaOpen Targets
0.10Weak
breast cancerOpen Targets
0.09Suggestive
subarachnoid hemorrhageOpen Targets
0.07Suggestive
cancerOpen Targets
0.06Suggestive
neoplasmOpen Targets
0.06Suggestive
prostate adenocarcinomaOpen Targets
0.06Suggestive
colitisOpen Targets
0.05Suggestive
hypopharyngeal carcinomaOpen Targets
0.04Suggestive
irritable bowel syndromeOpen Targets
0.03Suggestive
Cardiomyopathy, familial hypertrophicUniProt
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CALM3Protein interaction100%CALML3Protein interaction100%CALML5Protein interaction100%CALML4Protein interaction100%CALML6Protein interaction100%PRKACGProtein interaction99%
Tissue Expression6 tissues
Brain
100%
Bone Marrow
38%
Heart
23%
Lung
21%
Liver
11%
Ovary
10%
Gene Interaction Network
Click a node to explore
MYLK2CALM3CALML3CALML5CALML4CALML6PRKACG
PROTEIN STRUCTURE
Preparing viewer…
PDB3KF9 · 2.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.64LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.46 [0.34–0.64]
RankingsWhere MYLK2 stands among ~20K protein-coding genes
  • #8,343of 20,598
    Most Researched54
  • #4,518of 17,882
    Most Constrained (LOEUF)0.64
Genes detectedMYLK2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Multi-ancestry genome-wide association meta-analysis of Parkinson's disease.
PMID: 38155330
Nat Genet · 2024
1.00
2
FLNC and MYLK2 Gene Mutations in a Chinese Family with Different Phenotypes of Cardiomyopathy.
PMID: 33455984
Int Heart J · 2021
0.90
3
Mutational analysis of the kinase domain of MYLK2 gene in common human cancers.
PMID: 16448786
Pathol Res Pract · 2006
0.80
4
Differences in protein expression, at the basal state and at 2 h of insulin infusion, in muscle biopsies from healthy Arab men with high or low insulin sensitivity measured by hyperinsulinemic euglycemic clamp.
PMID: 36876056
Front Endocrinol (Lausanne) · 2022
0.70
5
[Relationship between electrocardiographic and genetic mutation (MYH7-H1717Q, MYLK2-K324E and KCNQ1-R190W) phenotype in patients with hypertrophic cardiomyopathy].
PMID: 26813553
Zhonghua Xin Xue Guan Bing Za Zhi · 2016
0.60