Biochemical Aspects of Cystic Fibrosis and Kidney Disease: A Brief Discussion

Abstract

Cystic fibrosis is a recessive autosomal disease. A nosographic entity that considered lethal in childhood. In organs such as the hypothalamus, kidney, and bone, the protein is directly affected, where its malfunction is likely to imply linear delays in growth, delayed pubertal onset, modulation of bone density, and susceptibility to renal calculus, which may be present in CF and have been in use for years, Nevertheless, the most researched and recognized cause of renal wound is iatrogenic in CF patients due to the consumption by pulmonary exacerbation of large doses of aminoglycosides (3-5 mg/kg/dose). The rise in the incidence of CF nephrocalcinosis does not seem to be connected to an inherent renal issue. Among fact, chronic kidney disease (CKD) prevalence in CF patients looks to be at least twice as high with a double rise every 10 years impacting over 20% of individuals older than 55 years. There has been no substantial change in proteome content in the treatment of CFTR modulators. These data indicate that a re-regulating proteasome activities adapts CF cells to the CFTR deficiency and impairs autophagy and endosomal aiming.