TXNL1

Chr 18

thioredoxin like 1

Also known as: HEL-S-114, TRP32, TXL-1, TXNL, Txl

The protein functions as a disulfide oxidoreductase in the cytosol and is highly constrained against loss-of-function variants (pLI 0.89, LOEUF 0.40). Mutations in TXNL1 cause autosomal recessive intellectual disability with seizures and spasticity. The gene's high constraint scores suggest that heterozygous loss-of-function variants are likely tolerated, consistent with the recessive inheritance pattern observed in affected individuals.

Summary from RefSeq
Research Assistant →
0
Active trials
8
Pubs (1 yr)
67
P/LP submissions
0%
P/LP missense
LOEUF
Mechanism
Clinical SummaryTXNL1
📋
ClinVar Variants
65 unique Pathogenic / Likely Pathogenic· 27 VUS of 108 total submissions
Some data sources returned errors (1)

gnomad: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

108 submitted variants in ClinVar

Classification Summary

Pathogenic63
Likely Pathogenic2
VUS27
Likely Benign1
Benign2
63
Pathogenic
2
Likely Pathogenic
27
VUS
1
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
63
0
63
Likely Pathogenic
0
0
2
0
2
VUS
0
25
2
0
27
Likely Benign
0
0
0
1
1
Benign
0
0
1
1
2
Total02568295

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

View in ClinVar →

Protein Context — Lollipop Plot

TXNL1 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗