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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
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SLC6A5
solute carrier family 6 member 5
Chromosome 11 Β· 11p15.1
NCBI Gene: 9152Ensembl: ENSG00000165970.12HGNC: HGNC:11051UniProt: Q4VAM4
41PubMed Papers
21Diseases
0Drugs
67Pathogenic Variants
FUNCTIONAL ROLE
Transporter
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
glycine:sodium symporter activitysynaptic transmission, glycinergicglycine import across plasma membraneplasma membranehyperekplexia 3hereditary hyperekplexiahyperekplexiaExaggerated startle response
✦AI Summary

SLC6A5 encodes glycine transporter 2 (GlyT2), a presynaptic sodium- and chloride-dependent transporter responsible for glycine reuptake and recycling at glycinergic synapses 1. GlyT2 is predominantly expressed in the brainstem, spinal cord, and cerebellum, where it maintains glycinergic neurotransmission by removing glycine from the synaptic cleft 1. Loss-of-function mutations in SLC6A5 impair glycine uptake and cause defective GlyT2 subcellular localization 2, leading to hyperekplexia 3, a rare neurogenetic disorder characterized by neonatal-onset exaggerated startle responses, muscle stiffness, and life-threatening apnea episodes 23. SLC6A5 mutations account for approximately 18.75% of genetically confirmed hyperekplexia cases 3. Animal models with Slc6a5 null alleles exhibit accelerated neuromuscular junction maturation and behavioral abnormalities including hyperactivity and altered grooming, phenotypes observed in schizophrenia models 4. Clinically, hyperekplexia caused by SLC6A5 mutations requires careful differential diagnosis from epilepsy, as antiepileptic drugs are contraindicated 5. Disease-associated mutations include missense, nonsense, and frameshift variants affecting predicted glycine and sodium binding sites 2.

Sources cited
1
GlyT2 is a Na+/Cl--dependent transporter expressed in brainstem, spinal cord, and cerebellum responsible for glycine uptake
PMID: 32712279
2
SLC6A5 mutations cause defective GlyT2 subcellular localization and decreased glycine uptake, resulting in hyperekplexia with neonatal excessive startle and apnea
PMID: 16751771
3
SLC6A5 mutations account for 18.75% of genetically confirmed hyperekplexia cases; hyperekplexia often misdiagnosed as epilepsy
PMID: 39051604
4
SLC6A5 mutations cause hyperekplexia 3; antiepileptic drugs should be avoided in this condition
PMID: 34797406
5
Slc6a5 mutations affect neuromuscular junction development and cause behavioral phenotypes including hyperactivity and altered grooming
PMID: 22272310
6
SLC6A5 mutations include missense, nonsense, and frameshift variants affecting glycine and Na+ binding sites
PMID: 16751771
Disease Associationsβ“˜21
hyperekplexia 3Open Targets
0.76Strong
hereditary hyperekplexiaOpen Targets
0.69Moderate
hyperekplexiaOpen Targets
0.48Moderate
Exaggerated startle responseOpen Targets
0.42Moderate
genetic disorderOpen Targets
0.42Moderate
brain diseaseOpen Targets
0.37Weak
cervicitisOpen Targets
0.29Weak
spondylolisthesisOpen Targets
0.27Weak
placenta praeviaOpen Targets
0.27Weak
DNA methylationOpen Targets
0.26Weak
diabetes mellitusOpen Targets
0.09Suggestive
attention deficit hyperactivity disorderOpen Targets
0.05Suggestive
autosomal recessive spondylocostal dysostosisOpen Targets
0.05Suggestive
hemoglobin D diseaseOpen Targets
0.05Suggestive
lung carcinomaOpen Targets
0.05Suggestive
Young adult-onset ParkinsonismOpen Targets
0.05Suggestive
hereditary attention deficit-hyperactivity disorderOpen Targets
0.04Suggestive
infantile-onset generalized dyskinesia with orofacial involvementOpen Targets
0.04Suggestive
congenital amegakaryocytic thrombocytopeniaOpen Targets
0.04Suggestive
congenital amegakaryocytic thrombocytopenia 1Open Targets
0.04Suggestive
Hyperekplexia 3UniProt
Pathogenic Variants67
NM_004211.5(SLC6A5):c.1315C>T (p.Arg439Ter)Pathogenic
Hyperekplexia 3|Inborn genetic diseases
β˜…β˜…β˜†β˜†2026β†’ Residue 439
NM_004211.5(SLC6A5):c.1430del (p.Ser477fs)Pathogenic
Hyperekplexia 3|not provided|Exaggerated startle response
β˜…β˜…β˜†β˜†2025β†’ Residue 477
NM_004211.5(SLC6A5):c.571C>T (p.Arg191Ter)Pathogenic
Hyperekplexia 3|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 191
NM_004211.5(SLC6A5):c.9C>A (p.Cys3Ter)Pathogenic
not provided|Hyperekplexia 3
β˜…β˜…β˜†β˜†2025β†’ Residue 3
NM_004211.5(SLC6A5):c.1131C>A (p.Tyr377Ter)Pathogenic
Hyperekplexia 3
β˜…β˜…β˜†β˜†2025β†’ Residue 377
NM_004211.5(SLC6A5):c.1640T>C (p.Phe547Ser)Pathogenic
Hyperekplexia 3|not provided|SLC6A5-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 547
NM_004211.5(SLC6A5):c.727C>A (p.Pro243Thr)Likely pathogenic
not provided|Hyperekplexia 3
β˜…β˜…β˜†β˜†2025β†’ Residue 243
NM_004211.5(SLC6A5):c.985+1G>ALikely pathogenic
Hyperekplexia 3|not provided
β˜…β˜…β˜†β˜†2025
NM_004211.5(SLC6A5):c.1286C>T (p.Pro429Leu)Pathogenic
Hyperekplexia 3
β˜…β˜…β˜†β˜†2024β†’ Residue 429
NM_004211.5(SLC6A5):c.2070+1G>APathogenic
Hyperekplexia 3
β˜…β˜…β˜†β˜†2024
NM_004211.5(SLC6A5):c.679+1G>APathogenic
Hyperekplexia 3
β˜…β˜…β˜†β˜†2024
NM_004211.5(SLC6A5):c.677del (p.Gly226fs)Pathogenic
Hyperekplexia 3
β˜…β˜…β˜†β˜†2024β†’ Residue 226
NM_004211.5(SLC6A5):c.323del (p.Pro108fs)Pathogenic
Hyperekplexia 3
β˜…β˜…β˜†β˜†2024β†’ Residue 108
NM_004211.5(SLC6A5):c.728C>G (p.Pro243Arg)Likely pathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2026β†’ Residue 243
NM_004211.5(SLC6A5):c.538_539del (p.Gln180fs)Pathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2025β†’ Residue 180
NM_004211.5(SLC6A5):c.621C>G (p.Tyr207Ter)Pathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2025β†’ Residue 207
NM_004211.5(SLC6A5):c.90C>A (p.Cys30Ter)Pathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2025β†’ Residue 30
NM_004211.5(SLC6A5):c.1969+1G>APathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2025
NM_004211.5(SLC6A5):c.1621C>T (p.Gln541Ter)Pathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2025β†’ Residue 541
NM_004211.5(SLC6A5):c.1181_1182delinsAA (p.Trp394Ter)Pathogenic
Hyperekplexia 3
β˜…β˜†β˜†β˜†2025β†’ Residue 394
View on ClinVar β†—
Related Genes
SLC17A6Protein interaction99%GAD1Protein interaction98%GPHNProtein interaction94%ARHGEF9Protein interaction90%SLC1A5Protein interaction88%SDCBPProtein interaction84%
Tissue Expression6 tissues
Brain
100%
Bone Marrow
11%
Lung
0%
Ovary
0%
Heart
0%
Liver
0%
Gene Interaction Network
Click a node to explore
SLC6A5SLC17A6GAD1GPHNARHGEF9SLC1A5SDCBP
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q4VAM4
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.76LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.59 [0.47–0.76]
RankingsWhere SLC6A5 stands among ~20K protein-coding genes
  • #10,089of 20,598
    Most Researched41
  • #1,093of 5,498
    Most Pathogenic Variants67 Β· top quartile
  • #6,044of 17,882
    Most Constrained (LOEUF)0.76
Genes detectedSLC6A5
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Myoclonus.
PMID: 24092290
Continuum (Minneap Minn) Β· 2013
1.00
2
SLC gene mutations and pediatric neurological disorders: diverse clinical phenotypes in a Saudi Arabian population.
PMID: 34797406
Hum Genet Β· 2022
0.90
3
Mutations in the gene encoding GlyT2 (SLC6A5) define a presynaptic component of human startle disease.
PMID: 16751771
Nat Genet Β· 2006
0.80
4
Hyperekplexia: A Single-Center Experience.
PMID: 39051604
J Child Neurol Β· 2024
0.70
5
Mutations within the human GLYT2 (SLC6A5) gene associated with hyperekplexia.
PMID: 16884688
Biochem Biophys Res Commun Β· 2006
0.60