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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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SLC6A17
solute carrier family 6 member 17
Chromosome 1 Β· 1p13.3
NCBI Gene: 388662Ensembl: ENSG00000197106.8HGNC: HGNC:31399UniProt: Q9H1V8
21PubMed Papers
21Diseases
0Drugs
2Pathogenic Variants
FUNCTIONAL ROLE
Transporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingmembranebrain developmentneutral amino acid transportprogressive essential tremor-speech impairment-facial dysmorphism-intellectual disability-abnormal behavior syndromeAbnormality of skin pigmentationactinic keratosisskin cancer
✦AI Summary

SLC6A17 is a synaptic vesicle transporter that mediates sodium-coupled, chloride-independent uptake of neutral amino acids, driven by the proton electrochemical gradient generated by vacuolar H(+)-ATPase 1. The transporter shows selectivity for proline, glycine, leucine, and alanine 1, and more recently glutamine has been identified as an endogenous substrate 2. SLC6A17 localizes to synaptic vesicles in glutamatergic and GABAergic neurons and likely contributes to neurotransmitter precursor uptake coupled to vesicle exocytosis. Pathogenic homozygous mutations in SLC6A17 cause autosomal-recessive intellectual developmental disorder 48 (IDRD48), characterized by moderate to severe intellectual disability with progressive tremor, speech impairment, and behavioral problems 3. Functional studies in knockout and mutant mice confirm learning deficits and establish decreased synaptic vesicle glutamine as a key pathogenic mechanism 2. Mutations cause either protein mislocalization or defective amino acid transport 32. Beyond neurological disease, SLC6A17 shows associations with skin pigmentation traits 4 and has emerged as a potential prognostic biomarker in lung adenocarcinoma, where elevated expression correlates with poorer survival outcomes 5. The gene also demonstrates dysregulation in preterm birth placentas 6.

Sources cited
1
SLC6A17 mediates glutamine transport into synaptic vesicles; mutations cause reduced vesicle glutamine and learning deficits in mice
PMID: 37440432
2
SLC6A17 functions as a synaptic vesicle transporter selective for proline, glycine, leucine, and alanine; transport is sodium-dependent and driven by vacuolar H(+)-ATPase gradient
PMID: 18768736
3
Homozygous SLC6A17 mutations cause autosomal-recessive intellectual disability with progressive tremor, speech impairment, and behavioral problems; mutations cause protein mislocalization or abnormal neuronal morphology
PMID: 25704603
4
SLC6A17 genetic variants are associated with facial skin color traits in Korean populations
PMID: 34648798
5
SLC6A17 expression is increased in lung adenocarcinoma and correlates with poorer overall, progression-free, and disease-specific survival
PMID: 39773040
6
SLC6A17 is among dysregulated genes in preterm birth placentas associated with trophoblast dysfunction
PMID: 36353514
Disease Associationsβ“˜21
progressive essential tremor-speech impairment-facial dysmorphism-intellectual disability-abnormal behavior syndromeOpen Targets
0.73Strong
Abnormality of skin pigmentationOpen Targets
0.39Weak
actinic keratosisOpen Targets
0.35Weak
skin cancerOpen Targets
0.33Weak
skin neoplasmOpen Targets
0.31Weak
squamous cell carcinomaOpen Targets
0.29Weak
generalized dystoniaOpen Targets
0.29Weak
sunburnOpen Targets
0.28Weak
acquired thrombocytopeniaOpen Targets
0.26Weak
basal cell carcinomaOpen Targets
0.25Weak
skin diseaseOpen Targets
0.25Weak
central nervous system cancerOpen Targets
0.04Suggestive
lung adenocarcinomaOpen Targets
0.04Suggestive
lung carcinomaOpen Targets
0.04Suggestive
glaucomaOpen Targets
0.03Suggestive
open-angle glaucomaOpen Targets
0.03Suggestive
small cell lung carcinomaOpen Targets
0.03Suggestive
glioblastoma multiformeOpen Targets
0.02Suggestive
astrocytomaOpen Targets
0.02Suggestive
intellectual disability, autosomal recessiveOpen Targets
0.01Suggestive
Intellectual developmental disorder, autosomal recessive 48UniProt
Pathogenic Variants2
NM_001010898.4(SLC6A17):c.484G>A (p.Gly162Arg)Pathogenic
Progressive essential tremor-speech impairment-facial dysmorphism-intellectual disability-abnormal behavior syndrome
β˜†β˜†β˜†β˜†2015β†’ Residue 162
NM_001010898.4(SLC6A17):c.1898C>G (p.Pro633Arg)Pathogenic
Progressive essential tremor-speech impairment-facial dysmorphism-intellectual disability-abnormal behavior syndrome
β˜†β˜†β˜†β˜†2015β†’ Residue 633
View on ClinVar β†—
Related Genes
UBL4BProtein interaction93%ALX3Protein interaction84%TEX15Protein interaction76%SLC6A15Shared pathway31%SLC6A20Shared pathway31%SLC38A5Shared pathway29%
Tissue Expression6 tissues
Brain
100%
Heart
0%
Ovary
0%
Liver
0%
Lung
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
SLC6A17UBL4BALX3TEX15SLC6A15SLC6A20SLC38A5
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q9H1V8
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.45Moderately Constrained
pLIβ“˜
1.00Intolerant
Observed/Expected LoF0.31 [0.22–0.45]
RankingsWhere SLC6A17 stands among ~20K protein-coding genes
  • #14,017of 20,598
    Most Researched21
  • #4,551of 5,498
    Most Pathogenic Variants2
  • #2,522of 17,882
    Most Constrained (LOEUF)0.45 Β· top quartile
Genes detectedSLC6A17
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Importance of glutamine in synaptic vesicles revealed by functional studies of
PMID: 37440432
Elife Β· 2023
1.00
2
Analysis of Breast Cancer Differences between China and Western Countries Based on Radiogenomics.
PMID: 36553681
Genes (Basel) Β· 2022
0.90
3
Homozygous SLC6A17 mutations cause autosomal-recessive intellectual disability with progressive tremor, speech impairment, and behavioral problems.
PMID: 25704603
Am J Hum Genet Β· 2015
0.80
4
GWAS Identifies Multiple Genetic Loci for Skin Color in Korean Women.
PMID: 34648798
J Invest Dermatol Β· 2022
0.70
5
Identification and functional characterization of solute carrier family 6 genes in Ciona savignyi.
PMID: 31026570
Gene Β· 2019
0.60