SLC52A3

Chr 20AR

solute carrier family 52 member 3

Also known as: BVVLS, BVVLS1, C20orf54, RFT2, RFVT3, bA371L19.1, hRFT2

This gene encodes a riboflavin transporter protein that facilitates intestinal absorption of riboflavin and localizes to the apical cell membrane with eleven transmembrane domains. Autosomal recessive mutations cause Brown-Vialetto-Van Laere syndrome 1 and Fazio-Londe disease through a dominant-negative mechanism. The protein's disrupted function leads to riboflavin deficiency affecting neuronal metabolism and survival.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.802 OMIM phenotypes
Clinical SummarySLC52A3
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Gene-Disease Validity (ClinGen)
Brown-Vialetto-van Laere syndrome 1 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.02) — 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
0.80LOEUF
pLI 0.019
Z-score 2.08
OE 0.38 (0.200.80)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.25Z-score
OE missense 0.79 (0.700.88)
214 obs / 271.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.38 (0.200.80)
00.351.4
Missense OE0.79 (0.700.88)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 5 / 13.1Missense obs/exp: 214 / 271.9Syn Z: -0.52
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveSLC52A3-related Brown-Vialetto-Van Laere syndromeLOFAR

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

DN
0.7326th %ile
GOF
0.6735th %ile
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

SLC52A3 · 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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