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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
TAPT1
transmembrane anterior posterior transformation 1
Chromosome 4 Β· 4p15.32
NCBI Gene: 202018Ensembl: ENSG00000169762.18HGNC: HGNC:26887UniProt: B4DJJ3
34PubMed Papers
21Diseases
0Drugs
9Pathogenic Variants
FUNCTIONAL ROLE
Receptor
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
positive regulation of cilium assemblycentrosomeciliary basal bodyendoplasmic reticulum membranecomplex lethal osteochondrodysplasiaosteochondrodysplasiaosteogenesis imperfectaconnective tissue disease
✦AI Summary

TAPT1 (transmembrane anterior posterior transformation 1) is a transmembrane protein that functions as a critical regulator of multiple developmental processes, particularly skeletal and neural development. The protein localizes to centrosomes and ciliary basal bodies, where it plays an essential role in ciliogenesis and primary cilium formation 1. TAPT1 acts as a negative regulator of BMP signaling by facilitating SMAD1/5 protein degradation through interaction with SMURF1 E3 ubiquitin ligase, thereby controlling osteogenic differentiation 2. Additionally, TAPT1 interacts with SUCO in the endoplasmic reticulum to maintain homeostasis of newly synthesized proteins crucial for brain development 3. Bi-allelic mutations in TAPT1 cause complex lethal osteochondrodysplasia (Symoens-Barnes-Gistelinck type), characterized by severe skeletal hypomineralization, intra-uterine fractures, and multi-organ developmental anomalies affecting brain, lungs, and kidneys 1. The phenotypic spectrum ranges from isolated early-onset cataract to severe syndromic disease 4. TAPT1 deficiency disrupts Golgi morphology, impairs ER-to-Golgi trafficking, and causes defective neural progenitor cell proliferation and differentiation, leading to microcephaly and motor dysfunction 3. The protein's role in maintaining cellular homeostasis makes it essential for proper embryonic development and adult tissue maintenance.

Sources cited
1
TAPT1 localizes to centrosomes and ciliary basal bodies, mutations cause complex lethal osteochondrodysplasia with skeletal and multi-organ defects
PMID: 26365339
2
TAPT1 inhibits BMP signaling by facilitating SMAD1/5 degradation through SMURF1 interaction
PMID: 36370851
3
TAPT1 interacts with SUCO in ER to maintain newly synthesized protein homeostasis and brain development
PMID: 41379998
4
TAPT1 mutations cause phenotypic spectrum ranging from early-onset cataract to severe syndromic disease
PMID: 36697720
⚠Limited data available β€” This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜21
complex lethal osteochondrodysplasiaOpen Targets
0.71Strong
osteochondrodysplasiaOpen Targets
0.67Moderate
osteogenesis imperfectaOpen Targets
0.37Weak
connective tissue diseaseOpen Targets
0.27Weak
diabetic ketoacidosisOpen Targets
0.26Weak
intracranial hemorrhageOpen Targets
0.25Weak
intelligenceOpen Targets
0.24Weak
eye diseaseOpen Targets
0.23Weak
cervical carcinomaOpen Targets
0.22Weak
mathematical abilityOpen Targets
0.19Weak
genetic disorderOpen Targets
0.19Weak
Abnormal nasolacrimal system morphologyOpen Targets
0.17Weak
crush injuryOpen Targets
0.16Weak
dislocationOpen Targets
0.14Weak
secondary malignant neoplasmOpen Targets
0.14Weak
thrombophiliaOpen Targets
0.14Weak
Recurrent fracturesOpen Targets
0.12Weak
Reduced bone mineral densityOpen Targets
0.12Weak
scoliosisOpen Targets
0.12Weak
Short statureOpen Targets
0.12Weak
Osteochondrodysplasia, complex lethal, Symoens-Barnes-Gistelinck typeUniProt
Pathogenic Variants9
NM_153365.3(TAPT1):c.917-1G>TLikely pathogenic
not provided
β˜…β˜†β˜†β˜†2025
NM_153365.3(TAPT1):c.806C>G (p.Ser269Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 269
NM_153365.3(TAPT1):c.846+2T>APathogenic
Complex lethal osteochondrodysplasia
β˜…β˜†β˜†β˜†2024
NM_153365.3(TAPT1):c.743_744del (p.Tyr248fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 248
NM_153365.3(TAPT1):c.1156C>T (p.Arg386Ter)Likely pathogenic
Complex lethal osteochondrodysplasia
β˜…β˜†β˜†β˜†2024β†’ Residue 386
NM_153365.3(TAPT1):c.1107+1_1107+8delLikely pathogenic
not provided
β˜…β˜†β˜†β˜†2023
NM_153365.3(TAPT1):c.199+1G>ALikely pathogenic
not provided
β˜…β˜†β˜†β˜†2022
NM_153365.3(TAPT1):c.1058A>T (p.Asp353Val)Pathogenic
Complex lethal osteochondrodysplasia
β˜†β˜†β˜†β˜†2018β†’ Residue 353
NM_153365.3(TAPT1):c.1108-1G>CPathogenic
Complex lethal osteochondrodysplasia
β˜†β˜†β˜†β˜†2018
View on ClinVar β†—
Related Genes
HOXC8Protein interaction81%CSTF1Protein interaction81%PPP1R35Shared pathway25%SAXO1Shared pathway20%ARB2AShared pathway20%KBTBD8Shared pathway17%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
42%
Liver
40%
Heart
29%
Lung
21%
Ovary
18%
Gene Interaction Network
Click a node to explore
TAPT1HOXC8CSTF1PPP1R35SAXO1ARB2AKBTBD8
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q6NXT6
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.70LoF Tolerant
pLIβ“˜
0.13Tolerant
Observed/Expected LoF0.44 [0.28–0.70]
RankingsWhere TAPT1 stands among ~20K protein-coding genes
  • #11,252of 20,598
    Most Researched34
  • #2,982of 5,498
    Most Pathogenic Variants9
  • #5,312of 17,882
    Most Constrained (LOEUF)0.70
Genes detectedTAPT1
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
A tapt1 knock-out zebrafish line with aberrant lens development and impaired vision models human early-onset cataract.
PMID: 36697720
Hum Genet Β· 2023
1.00
2
miR614 Expression Enhances Breast Cancer Cell Motility.
PMID: 33374314
Int J Mol Sci Β· 2020
0.90
3
Transmembrane anterior posterior transformation 1 regulates BMP signaling and modulates the protein stability of SMAD1/5.
PMID: 36370851
J Biol Chem Β· 2022
0.80
4
Genetic Defects in TAPT1 Disrupt Ciliogenesis and Cause a Complex Lethal Osteochondrodysplasia.
PMID: 26365339
Am J Hum Genet Β· 2015
0.70
5
A progeroid syndrome caused by a deep intronic variant in TAPT1 is revealed by RNA/SI-NET sequencing.
PMID: 36652330
EMBO Mol Med Β· 2023
0.60