Detection of misfolded protein aggregates from a clinical perspective

نویسندگان

  • Øyvind Halskau
  • Øyvind Strømland
  • Martin Jakubec
  • Samuel Furse
چکیده

List of abbreviations Alzheimer’s Disease, AD; Amyloid Precursor Protein, APP; Beta-Amyloid, Aβ; Bovine Spongiform Encephalopathy, BSE; Creutzfeldt-Jakob Disease, CJD; Central Nervous System, CNS; Immunofluorescence, IF; Immunoprecipitation, IP; Enzyme Linked Immunosorbent Assay, ELISA; Huntingtin, Htt; Huntington’s Disease, HD; Immunohistochemistry, IHC; Luminescent Conjugated Oligothiophenes, LCOs; Parkinson’s Disease, PD; Prion Protein Cellular, PrPC; Prion Protein Scrapie-associated, PrPSc; Protein Misfolding Disease, PMD; α-Synuclein, α-Syn; Western Blot, WB.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

The Deacetylase HDAC6 Regulates Aggresome Formation and Cell Viability in Response to Misfolded Protein Stress

The efficient clearance of cytotoxic misfolded protein aggregates is critical for cell survival. Misfolded protein aggregates are transported and removed from the cytoplasm by dynein motors via the microtubule network to a novel organelle termed the aggresome where they are processed. However, the means by which dynein motors recognize misfolded protein cargo, and the cellular factors that regu...

متن کامل

Characterization of conformation-specific, human-derived monoclonal antibodies against TTR aggregates with potential for diagnostic and therapeutic use

Misfolding and aggregation of transthyretin (TTR) is the basic pathophysiological mechanism of hereditary and wild type TTR amyloid (ATTR) amyloidosis. Polyneuropathy and/or cardiomyopathy with heart failure dominates the clinical presentation of the disease. Conformational changes of the TTR protein structure produce toxic intermediates that introduce cell death and ultimately loss of organ fu...

متن کامل

CK2 as anti-stress factor

Misfolded proteins are prone to form aggregates, which interfere with normal cellular functions. In general, the ubiquitin-proteasome system degrades such misfolded proteins to avoid aggregation. If this system becomes impaired or overloaded, an inclusion-body-like organelle, aggresome will operate. Misfolded protein aggregates are transported to aggresome with a deacetylase HDAC6 and dynein mo...

متن کامل

Protein Quality Control by Molecular Chaperones in Neurodegeneration

Protein homeostasis (proteostasis) requires the timely degradation of misfolded proteins and their aggregates by protein quality control (PQC), of which molecular chaperones are an essential component. Compared with other cell types, PQC in neurons is particularly challenging because they have a unique cellular structure with long extensions. Making it worse, neurons are postmitotic, i.e., cann...

متن کامل

Misfolded Superoxide Dismutase-1 In Sporadic and Familial Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative syndrome of unknown etiology that most commonly affects people in middle and high age. The hallmark of ALS is a progressive and simultaneous loss of upper and lower motor neurons in the central nervous system that leads to a progressive muscle atrophy, paralysis and death usually by respiratory failure. ALS is not a pure motor neu...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2016