CSTL1

Chr 20

cystatin like 1

Also known as: CTES1, RCET11, dJ322G13.4

The CSTL1 gene encodes a type 2 cystatin-like protein, though its specific function as a cysteine protease inhibitor has not been determined. Currently, no human diseases have been definitively associated with CSTL1 mutations in the medical literature.

Summary from RefSeq
Research Assistant →
0
Active trials
1
Pubs (1 yr)
21
P/LP submissions
0%
P/LP missense
1.92
LOEUF
DN
Mechanism· predicted
Clinical SummaryCSTL1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
21 unique Pathogenic / Likely Pathogenic· 47 VUS of 78 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.92LOEUF
pLI 0.000
Z-score -1.02
OE 1.42 (0.841.92)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.04Z-score
OE missense 1.01 (0.841.23)
77 obs / 76.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.42 (0.841.92)
00.351.4
Missense OE1.01 (0.841.23)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 10 / 7.1Missense obs/exp: 77 / 76.0Syn Z: 0.31
DN
0.6454th %ile
GOF
0.4973th %ile
LOF
0.3454th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

78 submitted variants in ClinVar

Classification Summary

Pathogenic18
Likely Pathogenic3
VUS47
Likely Benign5
18
Pathogenic
3
Likely Pathogenic
47
VUS
5
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
18
0
18
Likely Pathogenic
0
0
3
0
3
VUS
0
45
2
0
47
Likely Benign
0
4
1
0
5
Benign
0
0
0
0
0
Total04924073

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

CSTL1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗