SLC6A9

Chr 1ADAR

solute carrier family 6 member 9

Also known as: GCENSG, GLYT1, IS6

The protein is a sodium- and chloride-dependent glycine transporter that regulates glycine concentrations at inhibitory glycinergic synapses. Mutations cause glycine encephalopathy with normal serum glycine and increase susceptibility to isolated scoliosis. The gene shows both autosomal dominant and autosomal recessive inheritance patterns.

OMIMResearchSummary from OMIM, UniProt
LOFmechanismAD/ARLOEUF 0.632 OMIM phenotypes
Clinical SummarySLC6A9
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Gene-Disease Validity (ClinGen)
atypical glycine encephalopathy · 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
0.63LOEUF
pLI 0.000
Z-score 3.24
OE 0.40 (0.270.63)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.98Z-score
OE missense 0.74 (0.670.81)
331 obs / 449.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.40 (0.270.63)
00.351.4
Missense OE0.74 (0.670.81)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 14 / 34.6Missense obs/exp: 331 / 449.4Syn Z: 0.12
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongSLC6A9-related glycine encephalopathy with arthrogryposisLOFAR

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

DN
0.78top 25%
GOF
0.82top 10%
LOF
0.2287th %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

SLC6A9 · 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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