Reduced ELANE and SLPI expression compromises dental pulp cell activity.

ABSTRACT:

Background

Patients with ELANE variants and severe congenital neutropenia (SCN) commonly develop oral complications. Whether they are caused only by low neutrophil count or the combination of neutropenia and aberrant dental cells is unknown.

Methods

Genetic variant was identified with exome sequencing. Dental pulp cells isolated from the SCN patient with an ELANE mutation were investigated for gene expression, enzyme activity, proliferation, colony formation, wound healing, apoptosis, ROS, attachment, spreading and response to lipopolysaccharide.

Results

ELANE cells had diminished expression of ELANE and SLPI and reduced neutrophil elastase activity. Moreover, ELANE cells exhibited impaired proliferation, colony forming, migration, attachment and spreading; and signif

SUBMITTER: Sriwattanapong K 

PROVIDER: S-EPMC8560611 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature