Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation

Cystic fibrosis is a monogenic disease with a multisystemic phenotype, ranging from predisposition to chronic lung infection and inflammation to reduced bone mass. The exact mechanisms unbalancing the maintenance of an optimal bone mass in cystic fibrosis patients remain unknown. Multiple factors ma...

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Bibliographic Details
Published in:Biomolecules (Basel, Switzerland) Switzerland), 2023-02, Vol.13 (3), p.425
Main Authors: Fonseca, Óscar, Gomes, Maria Salomé, Amorim, Maria Adelina, Gomes, Ana Cordeiro
Format: Article
Language:English
Subjects:
bone
Bone density
Bone Diseases
Bone mass
Bronchopulmonary infection
Carrier proteins
CFTR
Chronic infection
Cystic fibrosis
Cystic Fibrosis - genetics
cystic fibrosis related bone disease
Cystic Fibrosis Transmembrane Conductance Regulator - genetics
Development and progression
Diabetes
FDA approval
Health aspects
Humans
Infections
Inflammation
Lung diseases
Medical screening
Mutation
Nutritional status
Osteoporosis
Pathophysiology
Phenotypes
Quality of Life
Regulatory approval
Review
Transplants & implants
Vitamin D
Vitamin deficiency
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Summary:Cystic fibrosis is a monogenic disease with a multisystemic phenotype, ranging from predisposition to chronic lung infection and inflammation to reduced bone mass. The exact mechanisms unbalancing the maintenance of an optimal bone mass in cystic fibrosis patients remain unknown. Multiple factors may contribute to severe bone mass reduction that, in turn, have devastating consequences in the patients' quality of life and longevity. Here, we will review the existing evidence linking the CFTR dysfunction and cell-intrinsic bone defects. Additionally, we will also address how the proinflammatory environment due to CFTR dysfunction in immune cells and chronic infection impairs the maintenance of an adequate bone mass in CF patients.
ISSN:2218-273X
2218-273X
DOI:10.3390/biom13030425