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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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SLC17A5
solute carrier family 17 member 5
Chromosome 6 Β· 6q13
NCBI Gene: 26503Ensembl: ENSG00000119899.14HGNC: HGNC:10933UniProt: Q9NRA2
53PubMed Papers
22Diseases
0Drugs
155Pathogenic Variants
FUNCTIONAL ROLE
Transporter
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
sialic acid transportD-glucuronate transmembrane transporter activitylysosomal membraneplasma membranefree sialic acid storage disease, infantile formSalla diseaseneurodegenerative diseaseintermediate severe Salla disease
✦AI Summary

SLC17A5 encodes sialin, a lysosomal membrane transporter responsible for exporting free sialic acid from lysosomes 1. The protein functions as a sialic acid:proton symporter and also transports D-glucuronate and other carbohydrate derivatives across the lysosomal membrane. Loss-of-function mutations in SLC17A5 cause free sialic acid storage disorders (FSASDs), including Salla disease and infantile sialic acid storage disorder, characterized by impaired lysosomal sialic acid efflux leading to cellular dysfunction and progressive neurological impairment 21. Beyond its primary role in lysosomal transport, emerging evidence implicates SLC17A5 in broader disease mechanisms. Genetic studies identified SLC17A5 variants as a novel Parkinson's disease susceptibility locus, with heterozygous carriers showing increased risk 3. Functional screening in Drosophila confirmed that knockdown of the SLC17A5 homolog enhances Ξ±-synuclein-induced neurodegeneration 4. Additionally, sialin mediates macrophage polarization and metabolic homeostasis through a sialin-cathepsin L-Nrf2 pathway, with dysregulation observed in metabolic dysfunction-associated steatohepatitis patients 5. Transcriptomic analyses identified SLC17A5 as differentially expressed between sexes in multiple sclerosis 6. Recent therapeutic advances include CRISPR base editing successfully correcting the founder FSASD variant c.115C>T with significant free sialic acid reduction 1.

Sources cited
1
SLC17A5 encodes sialin, a lysosomal transmembrane protein; loss causes free sialic acid storage; base editing corrects founder variant with FSA reduction
PMID: 37727271
2
SLC17A5 defects are a distinct subgroup of lysosomal storage disorders; clinical presentations are heterogeneous with neurodegenerative manifestations
PMID: 32389669
3
SLC17A5 is newly implicated as a candidate Parkinson's disease susceptibility gene with burden of rare damaging variants associated with PD risk
PMID: 29140481
4
SLC17A5 homolog knockdown enhances Ξ±-synuclein-induced progressive locomotor dysfunction in Drosophila, supporting role in neurodegeneration
PMID: 37200393
5
Sialin (encoded by SLC17A5) mediates macrophage rebalancing and metabolic homeostasis through sialin-cathepsin L-Nrf2 pathway; downregulated in MASH patients
PMID: 41034188
6
SLC17A5 identified as differentially expressed between males and females in multiple sclerosis brain tissue meta-analysis
PMID: 37023829
Disease Associationsβ“˜22
free sialic acid storage disease, infantile formOpen Targets
0.83Strong
Salla diseaseOpen Targets
0.82Strong
neurodegenerative diseaseOpen Targets
0.51Moderate
intermediate severe Salla diseaseOpen Targets
0.45Moderate
free sialic acid storage diseaseOpen Targets
0.44Moderate
genetic disorderOpen Targets
0.41Moderate
lysosomal storage diseaseOpen Targets
0.37Weak
hydrops fetalisOpen Targets
0.37Weak
inborn errors of metabolismOpen Targets
0.37Weak
chronic fatigue syndromeOpen Targets
0.28Weak
retroperitoneal cancerOpen Targets
0.15Weak
endometriosisOpen Targets
0.14Weak
type 2 diabetes mellitusOpen Targets
0.12Weak
diabetes mellitusOpen Targets
0.12Weak
focal segmental glomerulosclerosisOpen Targets
0.12Weak
enhanced S-cone syndromeOpen Targets
0.12Weak
Gitelman syndromeOpen Targets
0.12Weak
metabolic syndromeOpen Targets
0.11Weak
chronic kidney diseaseOpen Targets
0.11Weak
non-alcoholic fatty liver diseaseOpen Targets
0.11Weak
Infantile sialic acid storage disorderUniProt
Salla diseaseUniProt
Pathogenic Variants155
NM_012434.5(SLC17A5):c.918T>G (p.Tyr306Ter)Pathogenic
not specified|Salla disease|Salla disease;Sialic acid storage disease, severe infantile type|not provided|Sialic acid storage disease, severe infantile type
β˜…β˜…β˜†β˜†2026β†’ Residue 306
NM_012434.5(SLC17A5):c.115C>T (p.Arg39Cys)Pathogenic
Salla disease|not provided|Salla disease;Sialic acid storage disease, severe infantile type|Sialic acid storage disease, severe infantile type
β˜…β˜…β˜†β˜†2026β†’ Residue 39
NM_012434.5(SLC17A5):c.406A>G (p.Lys136Glu)Pathogenic
Salla disease|not provided|Salla disease;Sialic acid storage disease, severe infantile type|Sialic acid storage disease, severe infantile type
β˜…β˜…β˜†β˜†2026β†’ Residue 136
NM_012434.5(SLC17A5):c.802_816del (p.Ser268_Asn272del)Pathogenic
Sialic acid storage disease, severe infantile type|Salla disease|not provided|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2026β†’ Residue 268
NM_012434.5(SLC17A5):c.94+2T>ALikely pathogenic
Salla disease
β˜…β˜…β˜†β˜†2026
NM_012434.5(SLC17A5):c.144dup (p.Gly49fs)Pathogenic
Salla disease|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2026β†’ Residue 49
NM_012434.5(SLC17A5):c.204del (p.Asp69fs)Pathogenic
Salla disease
β˜…β˜…β˜†β˜†2026β†’ Residue 69
NM_012434.5(SLC17A5):c.116G>A (p.Arg39His)Pathogenic
Salla disease|not provided|Sialic acid storage disease, severe infantile type|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2026β†’ Residue 39
NM_012434.5(SLC17A5):c.507del (p.Ala169_Leu170insTer)Pathogenic
Salla disease|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2026β†’ Residue 169
NM_012434.5(SLC17A5):c.43G>T (p.Glu15Ter)Pathogenic
not provided|Salla disease|Salla disease;Sialic acid storage disease, severe infantile type
β˜…β˜…β˜†β˜†2026β†’ Residue 15
NM_012434.5(SLC17A5):c.719G>A (p.Trp240Ter)Pathogenic
Salla disease
β˜…β˜…β˜†β˜†2025β†’ Residue 240
NM_012434.5(SLC17A5):c.1260-2A>CPathogenic
Salla disease|Salla disease;Sialic acid storage disease, severe infantile type
β˜…β˜…β˜†β˜†2025
NM_012434.5(SLC17A5):c.1355_1356insAA (p.Val453fs)Pathogenic
Salla disease|Sialic acid storage disease, severe infantile type|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2025β†’ Residue 453
NM_012434.5(SLC17A5):c.979-2A>GLikely pathogenic
Salla disease
β˜…β˜…β˜†β˜†2025
NM_012434.5(SLC17A5):c.1138_1139del (p.Val380fs)Pathogenic
Salla disease|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 380
NM_012434.5(SLC17A5):c.291G>A (p.Thr97=)Pathogenic
Salla disease|not provided|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2025β†’ Residue 97
NM_012434.5(SLC17A5):c.667dup (p.Tyr223fs)Pathogenic
Salla disease|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2025β†’ Residue 223
NM_012434.5(SLC17A5):c.979-1G>TPathogenic
Salla disease|Salla disease;Sialic acid storage disease, severe infantile type
β˜…β˜…β˜†β˜†2025
NM_012434.5(SLC17A5):c.1259+1G>APathogenic
Sialic acid storage disease, severe infantile type|Salla disease|not provided|Colon adenocarcinoma|Familial cancer of breast
β˜…β˜…β˜†β˜†2025
NM_012434.5(SLC17A5):c.819+1G>APathogenic
Salla disease|not provided|Intermediate severe Salla disease|Sialic acid storage disease, severe infantile type;Salla disease
β˜…β˜…β˜†β˜†2025
View on ClinVar β†—
Related Genes
GNEProtein interaction85%SLC16A3Protein interaction78%LAMP1Protein interaction78%SLC16A7Protein interaction78%SLC16A8Protein interaction78%SLC22A10Shared pathway20%
Tissue Expression6 tissues
Liver
100%
Brain
97%
Lung
87%
Heart
61%
Bone Marrow
53%
Ovary
28%
Gene Interaction Network
Click a node to explore
SLC17A5GNESLC16A3LAMP1SLC16A7SLC16A8SLC22A10
PROTEIN STRUCTURE
Preparing viewer…
PDB8U3D Β· 2.83 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.06LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.80 [0.60–1.06]
RankingsWhere SLC17A5 stands among ~20K protein-coding genes
  • #8,495of 20,598
    Most Researched53
  • #493of 5,498
    Most Pathogenic Variants155 Β· top 10%
  • #10,735of 17,882
    Most Constrained (LOEUF)1.06
Genes detectedSLC17A5
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Excessive burden of lysosomal storage disorder gene variants in Parkinson's disease.
PMID: 29140481
Brain Β· 2017
1.00
2
Functional screening of lysosomal storage disorder genes identifies modifiers of alpha-synuclein neurotoxicity.
PMID: 37200393
PLoS Genet Β· 2023
0.90
3
SLC gene mutations and pediatric neurological disorders: diverse clinical phenotypes in a Saudi Arabian population.
PMID: 34797406
Hum Genet Β· 2022
0.80
4
Sodium nitrate protects against metabolic syndrome by sialin-mediated macrophage rebalance.
PMID: 41034188
Signal Transduct Target Ther Β· 2025
0.70
5
Base editing corrects the common Salla disease
PMID: 37727271
Mol Ther Nucleic Acids Β· 2023
0.60