Automated Author Profile

E., Tasdelen

Current S-Index

1.3

Sum of Dataset Indices for all datasets

Average Dataset Index per Dataset

0.7

Average Dataset Index per dataset

Total Datasets

2

Total datasets for this author

Average FAIR Score

84.6%

Average FAIR Score per dataset

Total Citations

2

Total citations to the author's datasets

Total Mentions

0

Total mentions of the author's datasets

S-Index Interpretation

S-Index Over Time

Cumulative Citations Over Time

Cumulative Mentions Over Time

Datasets

Supplementary Material for: Confirmation of the Hotspot Variant in MAP3K20 Responsible for Deafness, Ectodermal Dysplasia, Craniosynosytosis, Ectrodactyly and Skeletal Anomaly (DECES) Spectrum

Introduction: Heterozygous variants in MAP3K20 have been implicated in a recently identified syndromic form of ectodermal dysplasia, distinguished by a unique combination of ectrodactyly, craniosynostosis, bilateral sensorineural hearing loss, and skeletal anomalies such as transverse limb defect, brachydactyly.Case Presentation: We present an 11-year-old male with ectrodactyly, ectodermal dysplasia, bilateral sensorineural hearing loss, and cutaneous syndactyly in both hands. A de novo heterozygous variant, c.837_839del p.(Asn279del), in MAP3K20 was identified in his whole exome sequencing. Conclusion: The results of this study emphasize the critical role of MAP3K20 as a key gene in conditions involving ectrodactyly, craniosynostosis, bilateral sensorioneural hearing loss, ectodermal features, transverse limb defect and brachydactyly. We highlight the importance of prioritizing the recurrent p.(Asn279del) variant in genetic testing for affected individuals. Furthermore, we propose an acronym for this dominant disorder caused by the MAP3K20 gene vairants: DECES (Dominant/Deafness, Ectodermal Dysplasia, Craniosynostosis, Ectrodactyly, Skeletal anomalies).

Authors

  • karger, figshare admin ;
  • E., Tasdelen ;
  • M., Gönül ;
  • B., ÖztelcanGündüz ;
  • Ç., Üner ;
  • A., Büke ;
  • A., Sezer
1 Citation0 Mentions85% FAIR0.7 Dataset Index
10.6084/m9.figshare.295674082025

Supplementary Material for: Confirmation of the Hotspot Variant in MAP3K20 Responsible for Deafness, Ectodermal Dysplasia, Craniosynosytosis, Ectrodactyly and Skeletal Anomaly (DECES) Spectrum

Introduction: Heterozygous variants in MAP3K20 have been implicated in a recently identified syndromic form of ectodermal dysplasia, distinguished by a unique combination of ectrodactyly, craniosynostosis, bilateral sensorineural hearing loss, and skeletal anomalies such as transverse limb defect, brachydactyly.Case Presentation: We present an 11-year-old male with ectrodactyly, ectodermal dysplasia, bilateral sensorineural hearing loss, and cutaneous syndactyly in both hands. A de novo heterozygous variant, c.837_839del p.(Asn279del), in MAP3K20 was identified in his whole exome sequencing. Conclusion: The results of this study emphasize the critical role of MAP3K20 as a key gene in conditions involving ectrodactyly, craniosynostosis, bilateral sensorioneural hearing loss, ectodermal features, transverse limb defect and brachydactyly. We highlight the importance of prioritizing the recurrent p.(Asn279del) variant in genetic testing for affected individuals. Furthermore, we propose an acronym for this dominant disorder caused by the MAP3K20 gene vairants: DECES (Dominant/Deafness, Ectodermal Dysplasia, Craniosynostosis, Ectrodactyly, Skeletal anomalies).

Authors

  • karger, figshare admin ;
  • E., Tasdelen ;
  • M., Gönül ;
  • B., ÖztelcanGündüz ;
  • Ç., Üner ;
  • A., Büke ;
  • A., Sezer
1 Citation0 Mentions85% FAIR0.7 Dataset Index
10.6084/m9.figshare.29567408.v12025