Neurotoxicity Pathways in Drosophila Models of the Polyglutamine Disorders

2017 ◽  
pp. 201-223 ◽  
Author(s):  
M. Krench ◽  
J.T. Littleton
Keyword(s):  
Polyglutamine Disorders ◽  
Drosophila Models

Drosophila Models of Polyglutamine Disorders

2006 ◽  
pp. 587-594
Author(s):  
GEORGE R. JACKSON ◽  
TZU-KANG SANG ◽  
J. PAUL TAYLOR
Keyword(s):  
Polyglutamine Disorders ◽  
Drosophila Models

scholarly journals Autophagy Modulation as a Treatment of Amyloid Diseases

Molecules ◽  
2019 ◽  
Vol 24 (18) ◽  
pp. 3372 ◽  
Author(s):  
Zoe Mputhia ◽  
Eugene Hone ◽  
Timir Tripathi ◽  
Tim Sargeant ◽  
Ralph Martins ◽  
...  
Keyword(s):  
Protein Misfolding ◽  
Metabolic Diseases ◽  
Physiological Role ◽  
Cell Loss ◽  
Amyloid Proteins ◽  
Tissue Degeneration ◽  
Polyglutamine Disorders ◽  
Amyloid Accumulation ◽  
Causative Factors ◽  
Amyloid Diseases

Amyloids are fibrous proteins aggregated into toxic forms that are implicated in several chronic disorders. More than 30 diseases show deposition of fibrous amyloid proteins associated with cell loss and degeneration in the affected tissues. Evidence demonstrates that amyloid diseases result from protein aggregation or impaired amyloid clearance, but the connection between amyloid accumulation and tissue degeneration is not clear. Common examples of amyloid diseases are Alzheimer’s disease (AD), Parkinson’s disease (PD) and tauopathies, which are the most common forms of neurodegenerative diseases, as well as polyglutamine disorders and certain peripheral metabolic diseases. In these diseases, increased accumulation of toxic amyloid proteins is suspected to be one of the main causative factors in the disease pathogenesis. It is therefore important to more clearly understand how these toxic amyloid proteins accumulate as this will aide in the development of more effective preventive and therapeutic strategies. Protein homeostasis, or proteostasis, is maintained by multiple cellular pathways—including protein synthesis, quality control, and clearance—which are collectively responsible for preventing protein misfolding or aggregation. Modulating protein degradation is a very complex but attractive treatment strategy used to remove amyloid and improve cell survival. This review will focus on autophagy, an important clearance pathway of amyloid proteins, and strategies for using it as a potential therapeutic target for amyloid diseases. The physiological role of autophagy in cells, pathways for its modulation, its connection with apoptosis, cell models and caveats in developing autophagy as a treatment and as a biomarker is discussed.

scholarly journals Ku70 Alleviates Neurodegeneration in Drosophila Models of Huntington's Disease

PLoS ONE ◽  
2011 ◽  
Vol 6 (11) ◽  
pp. e27408 ◽  
Author(s):  
Takuya Tamura ◽  
Masaki Sone ◽  
Takeshi Iwatsubo ◽  
Kazuhiko Tagawa ◽  
Erich E. Wanker ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Drosophila Models

scholarly journals L-Carnitine in Drosophila: A Review

Antioxidants ◽  
2020 ◽  
Vol 9 (12) ◽  
pp. 1310
Author(s):  
Maria Rosaria Carillo ◽  
Carla Bertapelle ◽  
Filippo Scialò ◽  
Mario Siervo ◽  
Gianrico Spagnuolo ◽  
...  
Keyword(s):  
Transport System ◽  
Essential Role ◽  
Energy Homeostasis ◽  
Current Knowledge ◽  
Fatty Acyl ◽  
Amino Acid Derivative ◽  
Carnitine Transport ◽  
Human Transport ◽  
Drosophila Models

L-Carnitine is an amino acid derivative that plays a key role in the metabolism of fatty acids, including the shuttling of long-chain fatty acyl CoA to fuel mitochondrial β-oxidation. In addition, L-carnitine reduces oxidative damage and plays an essential role in the maintenance of cellular energy homeostasis. L-carnitine also plays an essential role in the control of cerebral functions, and the aberrant regulation of genes involved in carnitine biosynthesis and mitochondrial carnitine transport in Drosophila models has been linked to neurodegeneration. Drosophila models of neurodegenerative diseases provide a powerful platform to both unravel the molecular pathways that contribute to neurodegeneration and identify potential therapeutic targets. Drosophila can biosynthesize L-carnitine, and its carnitine transport system is similar to the human transport system; moreover, evidence from a defective Drosophila mutant for one of the carnitine shuttle genes supports the hypothesis of the occurrence of β-oxidation in glial cells. Hence, Drosophila models could advance the understanding of the links between L-carnitine and the development of neurodegenerative disorders. This review summarizes the current knowledge on L-carnitine in Drosophila and discusses the role of the L-carnitine pathway in fly models of neurodegeneration.

scholarly journals Enhanced sleep reverses memory deficits and underlying pathology in drosophila models of Alzheimer's disease

2017 ◽  
Vol 2 ◽  
pp. 15-26 ◽  
Author(s):  
Stephane Dissel ◽  
Markus Klose ◽  
Jeff Donlea ◽  
Lijuan Cao ◽  
Denis English ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Memory Deficits ◽  
Underlying Pathology ◽  
Drosophila Models
Sign in / Sign up

Export Citation Format

Share Document