TXNL4A

Chr 18

thioredoxin like 4A

Also known as: BMKS, DIB1, DIM1, SNRNP15, TXNL4, U5-15kD

The protein encoded by this gene is a member of the U5 small ribonucleoprotein particle (snRNP), and is involved in pre-mRNA splicing. This protein contains a thioredoxin-like fold and it is expected to interact with multiple proteins. Protein-protein interactions have been observed with the polyglutamine tract-binding protein 1 (PQBP1). Mutations in both the coding region and promoter region of this gene have been associated with Burn-McKeown syndrome, which is a rare disorder characterized by craniofacial dysmorphisms, cardiac defects, hearing loss, and bilateral choanal atresia. A pseudogene of this gene is found on chromosome 2. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Mar 2015]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtBurn-McKeown syndrome

Clinical highlights

Gene-disease validity (ClinGen)
choanal atresia-hearing loss-cardiac defects-craniofacial dysmorphism syndrome · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
0
Active trials
3
Pubs (1 yr)
P/LP submissions
P/LP missense
1.82
LOEUF
LOF
Mechanism· G2P
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GeneReview available — TXNL4A
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.82LOEUF
pLI 0.000
Z-score -0.15
OE 1.07 (0.571.82)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
2.77Z-score
OE missense 0.17 (0.110.26)
15 obs / 88.2 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?1.07 (0.571.82)
00.351.4
Missense OE?0.17 (0.110.26)
00.61.4
Synonymous OE?1.20
01.21.6
LoF obs/exp: 6 / 5.6Missense obs/exp: 15 / 88.2Syn Z: -0.89

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TXNL4A · 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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