Genetic heterogeneity in severe congenital neutropenia: how many aberrant pathways can kill a neutrophil?

AA Schäffer, C Klein - Current opinion in allergy and clinical …, 2007 - journals.lww.com
AA Schäffer, C Klein
Current opinion in allergy and clinical immunology, 2007journals.lww.com
We consider the numerous genes mutated in severe congenital neutropenia, the many
attempts to make animal models of severe congenital neutropenia, and the results from both
human and mouse studies investigating the molecular mechanisms of neutrophil apoptosis.
Investigations of how severe congenital neutropenia genes and apoptosis pathways are
connected should lead to a better understanding of the pathogenesis of neutropenia and
apoptosis pathways relevant to many cell types.
Summary
We consider the numerous genes mutated in severe congenital neutropenia, the many attempts to make animal models of severe congenital neutropenia, and the results from both human and mouse studies investigating the molecular mechanisms of neutrophil apoptosis. Investigations of how severe congenital neutropenia genes and apoptosis pathways are connected should lead to a better understanding of the pathogenesis of neutropenia and apoptosis pathways relevant to many cell types.
Lippincott Williams & Wilkins