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Related Experiment Video

Updated: Oct 18, 2025

Isolation, Propagation, and Prion Protein Expression During Neuronal Differentiation of Human Dental Pulp Stem Cells

Isolation, Propagation, and Prion Protein Expression During Neuronal Differentiation of Human Dental Pulp Stem Cells

Published on: March 18, 2019

Reduced ELANE and SLPI expression compromises dental pulp cell activity.

Kanokwan Sriwattanapong¹, Noppadol Sa-Ard-Iam², Lawan Boonprakong³

¹Genomics and Precision Dentistry Research Unit, Department of Physiology, Faculty of Dentistry, Chulalongkorn University, Bangkok, Thailand.

Cell Proliferation

|September 28, 2021

Summary

Severe congenital neutropenia (SCN) patients with ELANE mutations experience oral issues. Aberrant dental pulp cells, not just low neutrophils, contribute to these complications, impacting oral health.

Keywords:

cytokines inflammation neutropenia neutrophil elastase periodontitis protease

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Last Updated: Oct 18, 2025

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Published on: March 18, 2019

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Published on: January 12, 2017

Area of Science:

Genetics and Molecular Biology
Oral Medicine
Hematology

Background:

Severe congenital neutropenia (SCN) is linked to oral complications.
The exact cause, whether neutropenia alone or aberrant dental cells, remains unclear.

Purpose of the Study:

To investigate the role of dental pulp cells in oral complications of SCN patients with ELANE mutations.

Main Methods:

Exome sequencing identified the genetic variant.
Dental pulp cells from an SCN patient with an ELANE mutation were analyzed for gene expression, enzyme activity, cellular functions, and inflammatory responses.

Main Results:

ELANE mutation cells showed reduced ELANE/SLPI expression and neutrophil elastase activity.
These cells exhibited impaired proliferation, migration, and attachment, with increased ROS and apoptosis.
Inflammatory markers showed altered expression, with heightened responsiveness to LPS stimulation.

Conclusions:

Aberrant ELANE, SLPI, and downstream molecules in pulp cells contribute to oral complications in SCN.
Pulp cells with diminished neutrophil elastase and SLPI are highly sensitive to inflammation, exacerbating oral issues.