VSX1

Chr 20AD

visual system homeobox 1

Also known as: CAASDS, KTCN, KTCN1, PPCD, PPCD1, PPD, RINX

The protein encoded by this gene contains a paired-like homeodomain and binds to the core of the locus control region of the red/green visual pigment gene cluster. The encoded protein may regulate expression of the cone opsin genes early in development. Mutations in this gene can cause posterior polymorphous corneal dystrophy and keratoconus. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]

Primary Disease Associations & Inheritance

?Craniofacial anomalies and anterior segment dysgenesis syndromeMIM #614195
AD
Keratoconus 1MIM #148300
AD
0
Active trials
5
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.87
LOEUF
Mechanism
Clinical SummaryVSX1
Population Constraint (gnomAD)
Low constraint (pLI 0.04) — 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
0.87LOEUF
pLI 0.036
Z-score 1.85
OE 0.38 (0.190.87)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.48Z-score
OE missense 0.90 (0.801.02)
181 obs / 200.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.38 (0.190.87)
00.351.4
Missense OE0.90 (0.801.02)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 4 / 10.5Missense obs/exp: 181 / 200.2Syn Z: -0.92

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

VSX1 · protein map & ClinVar variants

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

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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC