Identification of a Novel TP63 Variant in a Chinese Patient with Orofacial Clefts and Ectrodactyly: Case Report and Literature Review

Author:

Jiang Leheng1ORCID,Jiang Chanyuan1,Song Tao1,Wang Yongqian1,Si Nuo1,Li Haidong1,Yin Ningbei1ORCID

Affiliation:

1. Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

Abstract

The TP63 gene is essential for epithelial proliferation, differentiation, and maintenance during embryogenesis. Despite considerable clinical variability, TP63-related symptoms are characterized by ectodermal dysplasia, distal limb malformations, and orofacial clefts. We identified a novel TP63 variant (c.619A > G, p.K207E) in a seven-month-old Chinese patient with orofacial clefts and ectrodactyly but no evident signs of ectodermal dysplasia. This phenotype was rarely reported before. We summarized the presence of the three main TP63-related manifestations in the literature and noted different distributions of CP- and CL/P-related variants regarding p63 structural domains.

Funder

CAMS Innovation Fund for Medical Sciences

2019 National Major Disease Multidisciplinary Diagnosis and Treatment Cooperation Project

2020 Science and Technology System Reform Project

Publisher

SAGE Publications

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