Protein homeostasis in neurons and its pathological alterations.
In neuronal cells, proteins are synthesized on ribosomes from the genetic information encoded in DNA. In some instances translation takes place at the neuronal cell soma but in other it occurs at distal location, such as in a dendritic spine. Folding is usually initiated before the completion of protein synthesis and its outcome strictly depends on the local environment in which the nascent protein is submerged. Incompletely folded proteins and, more importantly, misfolded proteins are under the surveillance of several quality control systems that re-establish the correct conformation or initiate protein degradation. Regulation and maintenance of these systems is a vital issue for neuronal and glial cells, and impairments at different levels leads to neurodegenerative diseases.
Malgaroli
Antonio
A
Scientific Institute San Raffaele, University Vita-Salute San Raffaele, Via Olgettina 58 20132 Milan, Italy. malgaroli.antonio@hsr.it
Vallar
Lucia
L
Zimarino
Vincenzo
V
D000682:Amyloid / Q000235:genetics / Q000378:metabolism; D000818:Animals; D001921:Brain / Q000378:metabolism* / Q000503:physiopathology; D006706:Homeostasis / Q000502:physiology; D006801:Humans; D002479:Inclusion Bodies / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000096:biosynthesis / Q000235:genetics / Q000378:metabolism*; D019636:Neurodegenerative Diseases / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology; D009474:Neurons / Q000378:metabolism* / Q000473:pathology; D011487:Protein Conformation; D017510:Protein Folding
D000682:Amyloid; D009419:Nerve Tissue Proteins