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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
SLC52A2
solute carrier family 52 member 2
Chromosome 8 Β· 8q24.3
NCBI Gene: 79581Ensembl: ENSG00000185803.12HGNC: HGNC:30224UniProt: A0A6Q8PHF8
40PubMed Papers
21Diseases
0Drugs
42Pathogenic Variants
FUNCTIONAL ROLE
ReceptorTransporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
plasma membraneriboflavin transmembrane transporter activity4-hydroxybutyrate receptor activityprotein bindingriboflavin transporter deficiencygenetic disorderhereditary sensory and autonomic neuropathyBrown-Vialetto-van Laere syndrome 1
✦AI Summary

SLC52A2 encodes RFVT2 (riboflavin transporter 2), a highly specific membrane protein essential for riboflavin (vitamin B2) distribution from blood to tissues 1. The transporter recognizes riboflavin through a conserved binding pocket and mediates its cellular uptake via a specific transport mechanism 2. Riboflavin is enzymatically converted to flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD), which serve as critical cofactors for oxidation-reduction reactions in carbohydrate, amino acid, and lipid metabolism 1. Mutations in SLC52A2 cause Riboflavin Transporter Deficiency (RTD), also known as Brown-Vialetto-Van Laere syndrome type 2, a rare childhood-onset neurodegenerative disorder characterized by pontobulbar palsy, sensorineural hearing loss, motor weakness, and respiratory symptoms 34. Retinal ganglion cells show particular vulnerability to RTD, resulting in vision loss 5. Early riboflavin supplementation significantly improves clinical outcomes including respiratory function, motor performance, and vision when initiated before symptom onset 54. Novel therapeutic approaches include AAV9-mediated gene augmentation therapy, which successfully rescues RTD motoneuron function in vitro 3. SLC52A2 expression is elevated in multiple human cancers and correlates with patient prognosis, particularly in hepatocellular carcinoma 6.

Sources cited
1
SLC52A2 mutations cause RTD type 2; AAV9-SLC52A2 gene therapy rescues motoneuron function
PMID: 40134705
2
SLC52A2-RTD presents with sensorineural hearing loss and vision loss; retinal ganglion cells are uniquely susceptible; early riboflavin supplementation improves vision acutely and preserves retinal structure
PMID: 35921603
3
SLC52A2 mutations cause Brown-Vialetto-Van Laere syndrome with bulbar palsy, hearing loss, and motor weakness; high-dose riboflavin improves respiratory and bulbar function
PMID: 31064337
4
RFVT2 (encoded by SLC52A2) mediates tissue distribution of riboflavin; riboflavin is converted to FMN and FAD for metabolic oxidation-reduction reactions; SLC52A2 mutations cause Brown-Vialetto-Van Laere syndrome
PMID: 34662687
5
RFVT2 recognizes riboflavin through a conserved binding pocket; provides structural basis for riboflavin recognition and transport mechanism
PMID: 40307217
6
SLC52A2 is highly expressed in multiple human cancers; expression correlates with patient survival and is an independent prognostic factor in hepatocellular carcinoma
PMID: 34991609
7
SLC52A2 mutations cause RTD with atypical presentations including sensory polyneuropathy; early riboflavin supplementation is important for preventing disease progression
PMID: 38965176
8
Cremophor EL inhibits SLC52A2-mediated riboflavin uptake, potentially contributing to paclitaxel-induced peripheral neuropathy
PMID: 31902922
Disease Associationsβ“˜21
riboflavin transporter deficiencyOpen Targets
0.83Strong
genetic disorderOpen Targets
0.52Moderate
hereditary sensory and autonomic neuropathyOpen Targets
0.46Moderate
Brown-Vialetto-van Laere syndrome 1Open Targets
0.46Moderate
Sensorineural hearing impairmentOpen Targets
0.44Moderate
sensorineural hearing lossOpen Targets
0.44Moderate
mitochondrial diseaseOpen Targets
0.43Moderate
auditory neuropathyOpen Targets
0.40Weak
optic atrophyOpen Targets
0.37Weak
cerebellar ataxiaOpen Targets
0.37Weak
neoplasmOpen Targets
0.12Weak
epidermolysis bullosa simplex with nail dystrophyOpen Targets
0.11Weak
breast cancerOpen Targets
0.10Weak
melanomaOpen Targets
0.10Suggestive
cancerOpen Targets
0.09Suggestive
esophageal squamous cell carcinomaOpen Targets
0.09Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.09Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
acute myeloid leukemiaOpen Targets
0.08Suggestive
nasopharyngeal carcinomaOpen Targets
0.08Suggestive
Brown-Vialetto-Van Laere syndrome 2UniProt
Pathogenic Variants42
NM_001363118.2(SLC52A2):c.383C>T (p.Ser128Leu)Pathogenic
Brown-Vialetto-van Laere syndrome 2|Inborn genetic diseases|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 128
NM_001363118.2(SLC52A2):c.808C>T (p.Gln270Ter)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided|Brown-Vialetto-van Laere syndrome 1
β˜…β˜…β˜†β˜†2025β†’ Residue 270
NM_001363118.2(SLC52A2):c.149dup (p.Tyr50Ter)Pathogenic
not provided|Brown-Vialetto-van Laere syndrome 2
β˜…β˜…β˜†β˜†2025β†’ Residue 50
NM_001363118.2(SLC52A2):c.1016T>C (p.Leu339Pro)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided|Inborn genetic diseases|SLC52A2-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 339
NM_001363118.2(SLC52A2):c.1030_1031del (p.Leu344fs)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 344
NM_001363118.2(SLC52A2):c.916G>A (p.Gly306Arg)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided|Inborn genetic diseases|Mitochondrial disease
β˜…β˜…β˜†β˜†2025β†’ Residue 306
NM_001363118.2(SLC52A2):c.1258G>A (p.Ala420Thr)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 420
NM_001363118.2(SLC52A2):c.935T>C (p.Leu312Pro)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided|Inborn genetic diseases
β˜…β˜…β˜†β˜†2025β†’ Residue 312
NM_001363118.2(SLC52A2):c.1088C>T (p.Pro363Leu)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜…β˜†β˜†2025β†’ Residue 363
NM_001363118.2(SLC52A2):c.1140del (p.Leu381fs)Pathogenic
not provided|Brown-Vialetto-van Laere syndrome 2
β˜…β˜…β˜†β˜†2025β†’ Residue 381
NM_001363118.2(SLC52A2):c.593G>A (p.Trp198Ter)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜…β˜†β˜†2023β†’ Residue 198
NM_001363118.2(SLC52A2):c.131-1G>CLikely pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜…β˜†β˜†2023
NM_001363118.2(SLC52A2):c.973T>G (p.Cys325Gly)Likely pathogenic
Sensorineural hearing loss disorder|Brown-Vialetto-van Laere syndrome 2
β˜…β˜…β˜†β˜†2020β†’ Residue 325
NM_001363118.2(SLC52A2):c.916G>C (p.Gly306Arg)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜†β˜†β˜†2026β†’ Residue 306
NM_001363118.2(SLC52A2):c.154T>C (p.Ser52Pro)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 52
NM_001363118.2(SLC52A2):c.155C>T (p.Ser52Phe)Pathogenic
Brown-Vialetto-van Laere syndrome 2|not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 52
NM_001363118.2(SLC52A2):c.551dup (p.Leu185fs)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜†β˜†β˜†2025β†’ Residue 185
NM_001363118.2(SLC52A2):c.545del (p.Gly182fs)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜†β˜†β˜†2024β†’ Residue 182
NM_001363118.2(SLC52A2):c.1076_1079del (p.Leu359fs)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜†β˜†β˜†2023β†’ Residue 359
NM_001363118.2(SLC52A2):c.543del (p.Gly182fs)Pathogenic
Brown-Vialetto-van Laere syndrome 2
β˜…β˜†β˜†β˜†2023β†’ Residue 182
View on ClinVar β†—
Related Genes
SLC52A1Shared pathway100%FFAR2Protein interaction70%SLC52A3Shared pathway67%SLC22A14Shared pathway50%RTBDNShared pathway50%FLAD1Shared pathway50%
Tissue Expression6 tissues
Lung
100%
Ovary
59%
Liver
59%
Brain
54%
Bone Marrow
53%
Heart
30%
Gene Interaction Network
Click a node to explore
SLC52A2SLC52A1FFAR2SLC52A3SLC22A14RTBDNFLAD1
PROTEIN STRUCTURE
Preparing viewer…
PDB8XSM Β· 3.01 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.95LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.64 [0.43–0.95]
RankingsWhere SLC52A2 stands among ~20K protein-coding genes
  • #10,238of 20,598
    Most Researched40
  • #1,496of 5,498
    Most Pathogenic Variants42
  • #8,971of 17,882
    Most Constrained (LOEUF)0.95
Genes detectedSLC52A2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Riboflavin transporter deficiency:
PMID: 40134705
Front Cell Neurosci Β· 2025
1.00
2
Ocular Biomarkers of Riboflavin Transporter Deficiency.
PMID: 35921603
J Neuroophthalmol Β· 2023
0.90
3
In Vitro Effects of Paclitaxel and Cremophor EL on Human Riboflavin Transporter SLC52A2.
PMID: 31902922
Biol Pharm Bull Β· 2020
0.80
4
Atypical presentations in an RTD patient and report of novel SLC52A3 and SLC52A2 mutations.
PMID: 38965176
Acta Neurol Belg Β· 2024
0.70
5
A Chinese pedigree with Brown-Vialetto-Van Laere syndrome due to two novel mutations of SLC52A2 gene: clinical course and response to riboflavin.
PMID: 31064337
BMC Med Genet Β· 2019
0.60