Search results for "chaperonopathy"
showing 4 items of 4 documents
Hsp60 as a Novel Target in IBD Management: A Prospect
2019
Inflammatory bowel disease (IBD) encompasses various pathological conditions similar but distinct that share a multifactorial etiology, including involvement of the intestinal barrier function, the immune system, and intestinal microorganisms. Hsp60 is a chaperonin component of the chaperoning system, present in all cells and tissues, including the intestine. It plays important roles in cell physiology outside and inside mitochondria, its canonical place of residence. However, Hsp60 can also be pathogenic in many conditions, the Hsp60 chaperonopathies, possibly including IBD. The various clinico-pathological types of IBD have a complicated mix of causative factors, among which Hsp60 can be …
Complex Destabilization in the Mitochondrial Chaperonin Hsp60 Leads to Disease.
2020
Several neurological disorders have been linked to mutations in chaperonin genes and more specifically to the HSPD1 gene. In humans, HSPD1 encodes for the mitochondrial Heat Shock Protein 60 (mtHsp60) chaperonin, which carries out essential protein refolding reactions that help maintain mitochondrial and cellular homeostasis. It functions as a macromolecular complex that provides client proteins an environment that favors proper folding in an ATP dependent manner. It has been established that mtHsp60 plays a crucial role in the proper folding of mitochondrial proteins involved in ATP producing pathways. Recently, various single-point mutations in the mtHsp60 encoding gene have been directly…
HSP-MOLECULAR CHAPERONES IN CANCER BIOGENESIS AND TUMOR THERAPY: AN OVERVIEW
2012
Molecular chaperones, many of which are heat-shock proteins (HSPs), are an important class of molecules with various functions. Pathological conditions in which chaperones become etiological and/or pathogenic factors are called chaperonopathies, and are classified into by defect, by excess, and by "mistake". In the latter case, the chaperone is structurally and functionally normal but paqrtecipates in pathwais that favor diseases, aòlthough in some cases the chaperone may have post-translational modifications that may lead it to change its location and function and, thus, to become pathogenic. For example, HSP-chaperones are involved in acrcinogenesis in various ways, so that some forms of …
ROLE OF CHAPERONES IN HEALTHY BOWEL AND IBD.
2015
The chaperoning system is the wole complement of chaperones, co-chaperones and chaperone cofactors of the body that preserves cell and tissue homeostasis. Its structural and/or functional defects can cause pathologic conditions, nemed chaperonopathies. Large bowel homeostasis includes a healthy status of the mucosal tissues and the microbiota. An alteration of one of them may determine, in turn, modifications of the other. Molecular chaperones of bacteria and human origin have been implicated in inflammatory bowel disease (IBD). In IBD chaperone levels usually increase and their cellular and subcellular loclization change. This is considered a physiological stress-response of mucosal cells …