Bridging human chaperonopathies and microbial chaperonins

• Published in: Communications biology Vol. 2; no. 1; p. 103
• Main Authors: Conway de Macario, Everly, Yohda, Masafumi, Macario, Alberto J. L., Robb, Frank T.
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 15-03-2019; Nature Publishing Group
• Subjects: 631/326/41/2537; 631/337/470/1981; 631/535; Archaeal Proteins - chemistry; Archaeal Proteins - genetics; Archaeal Proteins - metabolism; Bacterial Proteins - chemistry; Bacterial Proteins - genetics; Bacterial Proteins - metabolism; Biology; Biomedical and Life Sciences; Chaperones; Chaperonins; Chaperonins - chemistry; Chaperonins - genetics; Chaperonins - metabolism; Disease Susceptibility; Eukaryotic Cells - metabolism; Fungal Proteins; Humans; Life Sciences; Methanosarcinales - genetics; Methanosarcinales - metabolism; Molecular Chaperones - chemistry; Molecular Chaperones - genetics; Molecular Chaperones - metabolism; Molecular modelling; Mutation; Protein Conformation; Review; Review Article; Sulfolobales - genetics; Sulfolobales - metabolism
• ISSN: 2399-3642; 2399-3642
• DOI: 10.1038/s42003-019-0318-5

Chaperonins are molecular chaperones that play critical physiological roles, but they can be pathogenic. Malfunctional chaperonins cause chaperonopathies of great interest within various medical specialties. Although the clinical-genetic aspects of many chaperonopathies are known, the molecular mechanisms causing chaperonin failure and tissue lesions are poorly understood. Progress is necessary to improve treatment, and experimental models that mimic the human situation provide a promising solution. We present two models: one prokaryotic (the archaeon
Pyrococcus furiosus
) with eukaryotic-like chaperonins and one eukaryotic (
Chaetomium thermophilum
), both convenient for isolation-study of chaperonins, and report illustrative results pertaining to a pathogenic mutation of CCT5.
Everly Conway de Macario et al. present a review of microbial models of human chaperonopathies. They discuss the recent progress in using microbes to model human pathogenic mutations and to elucidate disease mechanisms.