SLC2A9

Chr 4ADAR

solute carrier family 2 member 9

Also known as: GLUT9, GLUTX, UAQTL2, URATv1

The protein functions as a high-capacity urate transporter involved in uric acid reabsorption by kidney proximal tubules, with residual glucose and fructose transport activity. Mutations cause hypouricemia, renal, 2, characterized by abnormally low serum uric acid levels due to impaired renal uric acid handling. Inheritance can be either autosomal dominant or autosomal recessive.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

{Uric acid concentration, serum, QTL 2}MIM #612076
ADAR
Hypouricemia, renal, 2MIM #612076
ADAR
UniProtHypouricemia renal 2
0
Active trials
29
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.14
LOEUF
Multiple*
Mechanism· predicted
Clinical SummarySLC2A9
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
1.14LOEUF
pLI 0.000
Z-score 1.05
OE 0.76 (0.521.14)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.12Z-score
OE missense 1.02 (0.931.12)
305 obs / 299.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.76 (0.521.14)
00.351.4
Missense OE1.02 (0.931.12)
00.61.4
Synonymous OE1.21
01.21.6
LoF obs/exp: 17 / 22.4Missense obs/exp: 305 / 299.0Syn Z: -1.90
DN
0.80top 10%
GOF
0.77top 25%
LOF
0.2092th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SLC2A9 · 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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