Alteration of protein folding and degradation in motor neuron diseases: Implications and protective functions of small heat shock proteins

Article ID	Journal	Published Year	Pages	File Type
6286591	Progress in Neurobiology	2012	18 Pages	PDF

Abstract

âº Several motor neuron diseases are associated to aberrant protein folding (misfolding). âº Protein misfolding results in aggregation as well as in the perturbation of several intracellular functions. âº Molecular chaperones, the proteasome and the autophagy are responsible for the clearance of misfolded proteins. âº Three members of the HSPB family have been found mutated in different motor neuron diseases. âº A member of the small heat shock protein (HSPB) family, HSPB8 facilitates the autophagic removal of misfolded proteins exerting protective role on motor neurons.

Keywords

DCTN1 autophagy-related gene 5 ATG5 CBP MND SOD1 PolyQ SBMA Charcot-Marie-Tooth lysosome-associated membrane protein 2a DRPLA FTLD TDP-43 OPTN CMT HMn HspB8 LAMP2A HDAC6 DHT GnRH UBQLN2 CMA dHMN SCA sequestosome1 NEFL NEDD8 SRC-1 SQSTM1 HSPs SALS Optineurin Chaperone-mediated autophagy Cu/Zn superoxide dismutase d-amino acid oxidase FUS/TLS spinocerebellar ataxia dentatorubral-pallidoluysian atrophy spinal and bulbar muscular atrophy Autophagy amyotrophic lateral sclerosis Sporadic amyotrophic lateral sclerosis familial amyotrophic lateral sclerosis ALS Huntington's disease Motor neuron diseases protein aggregation Neurodegeneration fALS DAO Dihydrotestosterone Distal hereditary motor neuropathy microtubule-associated protein light chain 3 ubiquitin–proteasome system Protein misfolding HIP neurofilament Hereditary motor neuropathy Gonadotropin-releasing hormone Hop Histone deacetylase 6 CREB-binding protein Small heat shock proteins Heat shock proteins Bag Androgen Receptor Steroid receptor coactivator 1 UPS