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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
CHRNB1
cholinergic receptor nicotinic beta 1 subunit
Chromosome 17 Β· 17p13.1
NCBI Gene: 1140Ensembl: ENSG00000170175.11HGNC: HGNC:1961UniProt: P11230
37PubMed Papers
22Diseases
22Drugs
34Pathogenic Variants
FUNCTIONAL ROLE
Ion ChannelReceptorTransporter
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
acetylcholine receptor activityprotein bindingchannel activitytransmitter-gated monoatomic ion channel activity involved in regulation of postsynaptic membrane potentialCongenital myasthenic syndromesmuscle crampMuscle spasmPostsynaptic congenital myasthenic syndromes
✦AI Summary

CHR17 encodes the beta-1 subunit of the muscle nicotinic acetylcholine receptor (AChR), a pentameric ion channel complex essential for neuromuscular transmission 1. Upon acetylcholine binding, the AChR undergoes conformational changes that open an ion-conducting channel, enabling cation transport across the postsynaptic membrane and muscle contraction. CHR17 is one of four genes (CHR17, CHR17, CHR17, CHR17) encoding the adult muscle AChR's transmembrane subunits 1. Pathogenic variants in CHR17 cause congenital myasthenic syndromes (CMS), characterized by impaired neuromuscular signal transmission presenting with early-onset feeding difficulties, ptosis, and limb weakness 1. Affected individuals respond to pyridostigmine and salbutamol therapy with satisfactory quality-of-life outcomes 1. CHR17 variants also contribute to autoimmune myasthenia gravis susceptibility; genome-wide association studies identified CHR17 as associated with late-onset myasthenia gravis, with rs4151121 identified as a potential causal variant 23. Additionally, CHR17 shows male-specific association with high myopia risk through effects on mitochondrial metabolism in ciliary tissues 4. Genetic studies are essential for accurate CMS diagnosis, as clinical and electrophysiological features alone are insufficient 5.

Sources cited
1
CHRNB1 encodes a transmembrane subunit of the muscle AChR pentamer; pathogenic variants cause congenital myasthenic syndromes with early onset and feeding difficulties; pyridostigmine and salbutamol are therapeutic options
PMID: 40768883
2
CHRNB1 has transcriptome-wide association with myasthenia gravis in skeletal muscle; genetic factors in AChR subunit genes contribute to myasthenia gravis pathogenesis
PMID: 35074870
3
CHRNB1 rs4151121 is a causal variant associated with late-onset myasthenia gravis risk, particularly in European populations
PMID: 40279038
4
CHRNB1 shows male-specific association with high myopia through effects on mitochondrial metabolism and respiratory chain complex assembly
PMID: 40081872
5
CHRNB1 variants cause congenital myasthenic syndromes; genetic studies are required for accurate diagnosis beyond clinical and electrophysiological features
PMID: 36835142
Disease Associationsβ“˜22
Congenital myasthenic syndromesOpen Targets
0.79Strong
muscle crampOpen Targets
0.53Moderate
Muscle spasmOpen Targets
0.53Moderate
Postsynaptic congenital myasthenic syndromesOpen Targets
0.46Moderate
congenital myasthenic syndrome, dominant/recessiveOpen Targets
0.40Weak
postsynaptic congenital myasthenic syndromeOpen Targets
0.37Weak
MyalgiaOpen Targets
0.32Weak
neurodegenerative diseaseOpen Targets
0.27Weak
acute respiratory distress syndromeOpen Targets
0.26Weak
strokeOpen Targets
0.26Weak
Limb painOpen Targets
0.23Weak
genetic disorderOpen Targets
0.19Weak
organophosphate poisoningOpen Targets
0.18Weak
osteoarthritis, hipOpen Targets
0.12Weak
diverticular diseaseOpen Targets
0.10Weak
medical procedureOpen Targets
0.09Suggestive
Abnormality of refractionOpen Targets
0.08Suggestive
prostate cancerOpen Targets
0.08Suggestive
PainOpen Targets
0.08Suggestive
BradycardiaOpen Targets
0.07Suggestive
Myasthenic syndrome, congenital, 2A, slow-channelUniProt
Myasthenic syndrome, congenital, 2C, associated with acetylcholine receptor deficiencyUniProt
Pathogenic Variants34
NM_000747.3(CHRNB1):c.727C>T (p.Arg243Cys)Pathogenic
not provided|Congenital myasthenic syndrome 2A
β˜…β˜…β˜†β˜†2026β†’ Residue 243
NM_000747.3(CHRNB1):c.1218-9_1218-7delPathogenic
not provided|Congenital myasthenic syndrome 2A
β˜…β˜…β˜†β˜†2026
NM_000747.3(CHRNB1):c.883del (p.Ala295fs)Pathogenic
Congenital myasthenic syndrome 2A|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 295
NM_000747.3(CHRNB1):c.865G>A (p.Val289Met)Pathogenic
Congenital myasthenic syndrome 2A|not provided|Congenital myasthenic syndrome 2C
β˜…β˜…β˜†β˜†2024β†’ Residue 289
NM_000747.3(CHRNB1):c.823G>T (p.Glu275Ter)Pathogenic
not provided|Congenital myasthenic syndrome 2A
β˜…β˜…β˜†β˜†2024β†’ Residue 275
NM_000747.3(CHRNB1):c.863del (p.Thr288fs)Pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2025β†’ Residue 288
NM_000747.3(CHRNB1):c.270G>A (p.Trp90Ter)Pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2024β†’ Residue 90
NM_000747.3(CHRNB1):c.610+1G>ALikely pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2024
NM_000747.3(CHRNB1):c.853C>A (p.Leu285Met)Pathogenic
Congenital myasthenic syndrome 2A|See cases
β˜…β˜†β˜†β˜†2024β†’ Residue 285
NM_000747.3(CHRNB1):c.544C>T (p.Gln182Ter)Likely pathogenic
Congenital myasthenic syndrome 2C;Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2024β†’ Residue 182
NM_000747.3(CHRNB1):c.434dup (p.Ile146fs)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 146
NM_000747.3(CHRNB1):c.757_820+135delLikely pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2023
NM_000747.3(CHRNB1):c.605dup (p.Ile203fs)Pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2023β†’ Residue 203
GRCh38/hg38 17p13.1(chr17:7454200-7454618)x0Pathogenic
Congenital myasthenic syndrome 2C
β˜…β˜†β˜†β˜†2023
NM_000747.3(CHRNB1):c.1347_1355del (p.Glu449_Glu451del)Pathogenic
Congenital myasthenic syndrome 2C|Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2022β†’ Residue 449
NM_000747.3(CHRNB1):c.1071C>A (p.Tyr357Ter)Likely pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2022β†’ Residue 357
NC_000017.11:g.(?_7454277)_(7454540_?)delPathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2022
NM_000747.3(CHRNB1):c.1072_1073del (p.Leu358fs)Pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2022β†’ Residue 358
NM_000747.3(CHRNB1):c.1102_1103del (p.Asp368fs)Pathogenic
Congenital myasthenic syndrome 2A
β˜…β˜†β˜†β˜†2022β†’ Residue 368
NM_000747.3(CHRNB1):c.42_44delinsAA (p.Ala15fs)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2022β†’ Residue 15
View on ClinVar β†—
Drug Targets22
ATRACURIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
ATRACURIUM BESYLATEEARLY_PHASE_1
Muscle-type nicotinic acetylcholine receptor antagonist
CISATRACURIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
Muscle spasm
CISATRACURIUM BESYLATEPhase III
Muscle-type nicotinic acetylcholine receptor antagonist
acute respiratory distress syndrome
DECAMETHONIUMApproved
Muscle-type nicotinic acetylcholine receptor partial agonist
Muscle spasm
DOXACURIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
muscle cramp
GALLAMINE TRIETHIODIDEApproved
Muscle-type nicotinic acetylcholine receptor antagonist
METOCURINE IODIDEApproved
Muscle-type nicotinic acetylcholine receptor antagonist
MIVACURIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
MIVACURIUM CHLORIDEPhase II
Muscle-type nicotinic acetylcholine receptor antagonist
Decreased liver function
PANCURONIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
PIPECURONIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
muscle cramp
PIPECURONIUM BROMIDEUNKNOWN
Muscle-type nicotinic acetylcholine receptor antagonist
RAPACURONIUM BROMIDEApproved
Muscle-type nicotinic acetylcholine receptor antagonist
Muscle spasm
ROCURONIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
Muscle spasm
ROCURONIUM BROMIDEPhase III
Muscle-type nicotinic acetylcholine receptor antagonist
SUCCINYLCHOLINE CHLORIDEApproved
Muscle-type nicotinic acetylcholine receptor agonist
SUXAMETHONIUMApproved
Muscle-type nicotinic acetylcholine receptor agonist
muscle cramp
TUBOCURARINEApproved
Muscle-type nicotinic acetylcholine receptor antagonist
TUBOCURARINE CHLORIDEUNKNOWN
Muscle-type nicotinic acetylcholine receptor antagonist
VECURONIUMApproved
Muscle-type nicotinic acetylcholine receptor antagonist
muscle cramp
VECURONIUM BROMIDEApproved
Muscle-type nicotinic acetylcholine receptor antagonist
Related Genes
RAPSNProtein interaction93%CHRNA7Protein interaction91%CHRNEProtein interaction78%CHRNB4Protein interaction77%MUSKProtein interaction77%CHRNGProtein interaction75%
Tissue Expression6 tissues
Liver
100%
Heart
62%
Bone Marrow
51%
Brain
41%
Lung
31%
Ovary
25%
Gene Interaction Network
Click a node to explore
CHRNB1RAPSNCHRNA7CHRNECHRNB4MUSKCHRNG
PROTEIN STRUCTURE
Preparing viewer…
PDB9DMS Β· 1.92 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.05LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.82 [0.65–1.05]
RankingsWhere CHRNB1 stands among ~20K protein-coding genes
  • #10,603of 20,598
    Most Researched37
  • #99of 1,025
    FDA-Approved Drug Targets16 Β· top 10%
  • #1,710of 5,498
    Most Pathogenic Variants34
  • #10,458of 17,882
    Most Constrained (LOEUF)1.05
Genes detectedCHRNB1
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Clinical and Pathologic Features of Congenital Myasthenic Syndromes Caused by 35 Genes-A Comprehensive Review.
PMID: 36835142
Int J Mol Sci Β· 2023
1.00
2
A 20-year Clinical and Genetic Neuromuscular Cohort Analysis in Lebanon: An International Effort.
PMID: 34602496
J Neuromuscul Dis Β· 2022
0.90
3
Neurogenetic fetal akinesia and arthrogryposis: genetics, expanding genotype-phenotypes and functional genomics.
PMID: 33060286
J Med Genet Β· 2021
0.80
4
Identification of genetic risk loci and prioritization of genes and pathways for myasthenia gravis: a genome-wide association study.
PMID: 35074870
Proc Natl Acad Sci U S A Β· 2022
0.70
5
Causal Variants in CHRNA1 and CHRNB1 Genes for Anti-acetylcholine Receptor Antibody Positive Myasthenia Gravis: Evidence from Bayesian Fine-Mapping and Genetic Association Study.
PMID: 40279038
Mol Neurobiol Β· 2025
0.60