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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
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TXNDC15
thioredoxin domain containing 15
Chromosome 5 Β· 5q31.1
NCBI Gene: 79770Ensembl: ENSG00000113621.16HGNC: HGNC:20652UniProt: Q96J42
28PubMed Papers
21Diseases
0Drugs
7Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingpositive regulation of smoothened signaling pathwaycilium assemblyciliummeckel syndrome 14Meckel syndromeciliopathyMungan syndrome
✦AI Summary

TXNDC15 is a thioredoxin domain-containing transmembrane protein primarily localized to the endoplasmic reticulum that serves critical functions in ciliary biology and homeostasis 1. It acts as a positive regulator of ciliary hedgehog signaling and is essential for ciliogenesis 2. Mechanistically, TXNDC15 maintains transition zone (TZ) integrity at the ciliary base through its thioredoxin domain, which contains functionally important cysteine residues 1. Loss of TXNDC15 function impairs MKS module assembly and disrupts ciliary membrane protein localization 1. Clinically, TXNDC15 mutations cause Meckel syndrome 14 (MKS14), a perinatally lethal autosomal recessive ciliopathy characterized by occipital encephalocele, polycystic kidneys, and postaxial polydactyly 2, 3, 4, 5. Multiple pathogenic variants including loss-of-function mutations and compound heterozygous alleles have been identified in affected families 3, 4, 5. Beyond ciliopathies, emerging proteomic evidence suggests TXNDC15 may have ancestry-specific associations with Parkinson's disease risk in European populations and influences adiposity-related pathways 6, 7, though these associations require further mechanistic validation.

Sources cited
1
TXNDC15 identified as novel morbid gene encoding thiol isomerase with loss-of-function mutations in Meckel-Gruber syndrome; involved in ciliogenesis and ciliary signaling
PMID: 27894351
2
TXNDC15 bi-allelic variants confirmed as cause of Meckel syndrome in fetal cases; validates TXNDC15 causation of MKS
PMID: 31411728
3
TXNDC15 is ER-localized thioredoxin-like transmembrane protein that maintains transition zone integrity; thioredoxin domain cysteine residues are functionally critical
PMID: 39679447
4
Novel homozygous TXNDC15 mutation (c.560delA) causes Meckel syndrome 14 in Chinese population
PMID: 38156946
5
TXNDC15 compound heterozygous mutations cause Meckel-Gruber syndrome; first non-consanguineous family report with MKS due to TXNDC15
PMID: 30851085
6
TXNDC15 identified as ancestry-specific causal protein associated with Parkinson's disease risk in Europeans
PMID: 41504309
7
TXNDC15 identified through bidirectional MR analysis as protein affecting BMI levels in adiposity pathways
PMID: 37676424
Disease Associationsβ“˜21
meckel syndrome 14Open Targets
0.74Strong
Meckel syndromeOpen Targets
0.58Moderate
ciliopathyOpen Targets
0.37Weak
Mungan syndromeOpen Targets
0.37Weak
neurodegenerative diseaseOpen Targets
0.31Weak
genetic disorderOpen Targets
0.19Weak
severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-positiveOpen Targets
0.12Weak
Menkes diseaseOpen Targets
0.03Suggestive
ovarian carcinomaOpen Targets
0.02Suggestive
Joubert syndromeOpen Targets
0.01Suggestive
Parkinson diseaseOpen Targets
0.01Suggestive
visceral LeishmaniasisOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
glioblastoma multiformeOpen Targets
0.00Suggestive
holoprosencephalyOpen Targets
0.00Suggestive
colorectal carcinomaOpen Targets
0.00Suggestive
cerebral creatine deficiency syndromeOpen Targets
0.00Suggestive
cystOpen Targets
0.00Suggestive
ImmunodeficiencyOpen Targets
0.00Suggestive
Meckel syndrome 14UniProt
Pathogenic Variants7
NM_024715.4(TXNDC15):c.211dup (p.Gln71fs)Pathogenic
Meckel syndrome 14|not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 71
NM_024715.4(TXNDC15):c.673_687del (p.Ser225_His229del)Pathogenic
Meckel-Gruber syndrome|Meckel syndrome 14
β˜…β˜†β˜†β˜†2024β†’ Residue 225
NM_024715.4(TXNDC15):c.103+1G>APathogenic
Meckel-Gruber syndrome|Meckel syndrome 14
β˜…β˜†β˜†β˜†2024
NM_024715.4(TXNDC15):c.379C>T (p.Arg127Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2020β†’ Residue 127
NM_024715.4(TXNDC15):c.844C>T (p.Arg282Ter)Pathogenic
Meckel-Gruber syndrome|Meckel syndrome 14
β˜…β˜†β˜†β˜†β†’ Residue 282
NM_024715.4(TXNDC15):c.956dup (p.Ser321fs)Pathogenic
Meckel syndrome 14
β˜†β˜†β˜†β˜†2022β†’ Residue 321
NM_024715.4(TXNDC15):c.635T>C (p.Leu212Pro)Pathogenic
Meckel syndrome 14
β˜†β˜†β˜†β˜†2022β†’ Residue 212
View on ClinVar β†—
Related Genes
ARMC9Shared pathway100%CIBAR2Shared pathway50%CFAP184Shared pathway50%HYLS1Shared pathway50%LRRC45Shared pathway50%WDR90Shared pathway50%
Tissue Expression6 tissues
Heart
100%
Brain
93%
Ovary
69%
Lung
63%
Liver
54%
Bone Marrow
19%
Gene Interaction Network
Click a node to explore
TXNDC15ARMC9CIBAR2CFAP184HYLS1LRRC45WDR90
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q96J42
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.97LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.69 [0.50–0.97]
RankingsWhere TXNDC15 stands among ~20K protein-coding genes
  • #12,478of 20,598
    Most Researched28
  • #3,256of 5,498
    Most Pathogenic Variants7
  • #9,283of 17,882
    Most Constrained (LOEUF)0.97
Genes detectedTXNDC15
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Characterizing the morbid genome of ciliopathies.
PMID: 27894351
Genome Biol Β· 2016
1.00
2
Meckel syndrome: Clinical and mutation profile in six fetuses.
PMID: 31411728
Clin Genet Β· 2019
0.90
3
TXNDC15, an ER-localized thioredoxin-like transmembrane protein, contributes to ciliary transition zone integrity.
PMID: 39679447
J Cell Sci Β· 2024
0.80
4
Novel homozygous mutations in TXNDC15 causing Meckel syndrome.
PMID: 38156946
Mol Genet Genomic Med Β· 2024
0.70
5
Proteomic and Genetic Insights into Ancestry-Specific Associations in Parkinson's Disease.
PMID: 41504309
Mov Disord Β· 2026
0.60