SLC52A2

Chr 8AR

solute carrier family 52 member 2

Also known as: BVVLS2, D15Ertd747e, GPCR41, GPR172A, HuPAR-1, PAR1, RFT3, RFVT2

This protein transports riboflavin (vitamin B2) across cell membranes, particularly in brain tissue, where riboflavin is processed into essential coenzymes FMN and FAD for cellular metabolism. Mutations cause Brown-Vialetto-Van Laere syndrome 2, an autosomal recessive progressive neurologic disorder characterized by deafness, bulbar dysfunction, and axial and limb hypotonia. The pathogenic mechanism involves gain-of-function mutations that disrupt normal riboflavin transport.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.971 OMIM phenotype
Clinical SummarySLC52A2
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Gene-Disease Validity (ClinGen)
Brown-Vialetto-van Laere syndrome 2 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.97LOEUF
pLI 0.002
Z-score 1.63
OE 0.49 (0.270.97)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.85Z-score
OE missense 1.15 (1.041.26)
300 obs / 261.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.49 (0.270.97)
00.351.4
Missense OE1.15 (1.041.26)
00.61.4
Synonymous OE1.35
01.21.6
LoF obs/exp: 6 / 12.1Missense obs/exp: 300 / 261.5Syn Z: -3.13
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveSLC52A2-related Brown-Vialetto-Van Laere syndromeLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7230th %ile
GOF
0.76top 25%
LOF
0.2289th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SLC52A2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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