Abnormal primary and permanent dentitions with ectodermal symptoms predict WNT10A deficiency.

ABSTRACT:

Background

The WNT10A protein is critical for the development of ectodermal appendages. Variants in the WNT10A gene may be associated with a spectrum of ectodermal abnormalities including extensive tooth agenesis.

Methods

In seven patients with severe tooth agenesis we identified anomalies in primary dentition and additional ectodermal symptoms, and assessed WNT10A mutations by genetic analysis.

Results

Investigation of primary dentition revealed peg-shaped crowns of primary mandibular incisors and three individuals had agenesis of at least two primary teeth. The permanent dentition was severely affected in all individuals with a mean of 21 missing teeth. Primary teeth were most often present in positions were succedaneous teeth were missing. Furthermore, most existin

SUBMITTER: Bergendal B 

PROVIDER: S-EPMC5122154 | biostudies-literature | 2016 Nov

REPOSITORIES: biostudies-literature