Book ChapterDOI
Molecular and Cellular Insights: Neuroinflammation and Amyotrophic Lateral Sclerosis
Arun Upadhyay,Ayeman Amanullah,Vibhuti Joshi,Ribhav Mishra,Amit Mishra +4 more
- pp 209-230
TLDR
This chapter will focus on the precise understanding of neuroinflammatory responses in molecular pathomechanism of ALS and it also discusses new potential therapeutic strategies to improve neuroprotection and to alleviate proteotoxicity in ALS linked motor neurodegeneration.Abstract:
Accumulation of misfolded and abnormal proteins generates probably a common and complex pathomechanism in various neurodegenerative diseases (Alzheimer’s, Parkinson’s, Huntington’s, Amyotrophic Lateral Sclerosis, and Prion) and in aging. In amyotrophic lateral sclerosis (ALS), neuroinflammation appears in the form of T-lymphocyte infiltration, presence of reactive astroglial and microglial cells. Most likely, end stage of this toxic cascade results in death of motor neurons in the cortex, brainstem, and spinal cord. More than 10 different genetic causes of familial ALS are known; but still it is a challenge to prevent the loss of descending motor tracts by suppressing the degeneration of motor neurons. This chapter will focus on the precise understanding of neuroinflammatory responses in molecular pathomechanism of ALS and it also discusses new potential therapeutic strategies to improve neuroprotection and to alleviate proteotoxicity in ALS linked motor neurodegeneration.read more
Citations
More filters
Journal ArticleDOI
Amyloids of multiple species: are they helpful in survival?
Arun Upadhyay,Amit Mishra +1 more
TL;DR: This review aims to extend current understanding about the multifaceted useful properties of amyloids and their functional interactions with other molecular pathways in various species to provide new insights to identify novel therapeutic strategies for ageing and neurodegenerative diseases.
References
More filters
Journal ArticleDOI
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
TL;DR: Tight genetic linkage between FALS and a gene that encodes a cytosolic, Cu/Zn-binding superoxide dismutase (SOD1), a homodimeric metalloenzyme that catalyzes the dismutation of the toxic superoxide anion O–2 to O2 and H2O2 is reported.
Journal ArticleDOI
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann,Deepak M. Sampathu,Linda K. Kwong,Adam C. Truax,Matthew Micsenyi,Thomas T. Chou,Jennifer Bruce,Theresa Schuck,Murray Grossman,Christopher M. Clark,Leo McCluskey,Bruce L. Miller,Eliezer Masliah,Ian R. A. Mackenzie,Howard Feldman,Wolfgang Feiden,Hans A. Kretzschmar,John Q. Trojanowski,Virginia M.-Y. Lee +18 more
TL;DR: It is shown that TDP-43 is the major disease protein in both frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis.
Journal ArticleDOI
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS
Mariely DeJesus-Hernandez,Ian R. A. Mackenzie,Bradley F. Boeve,Adam L. Boxer,Matt Baker,Nicola J. Rutherford,Alexandra M. Nicholson,Ni Cole A. Finch,Heather C. Flynn,Jennifer Adamson,Naomi Kouri,Aleksandra Wojtas,Pheth Sengdy,Ging-Yuek Robin Hsiung,Anna Karydas,William W. Seeley,Keith A. Josephs,Giovanni Coppola,Daniel H. Geschwind,Zbigniew K. Wszolek,Howard Feldman,Howard Feldman,David S. Knopman,Ronald C. Petersen,Bruce L. Miller,Dennis W. Dickson,Kevin B. Boylan,Neill R. Graff-Radford,Rosa Rademakers +28 more
TL;DR: It is found that repeat expansion in C9ORF72 is a major cause of both FTD and ALS, suggesting multiple disease mechanisms.
Journal ArticleDOI
The inflammatory reflex
TL;DR: The discovery that cholinergic neurons inhibit acute inflammation has qualitatively expanded understanding of how the nervous system modulates immune responses, and the opportunity now exists to apply this insight to the treatment of inflammation through selective and reversible 'hard-wired' neural systems.
Journal ArticleDOI
Glutamate Receptor Ion Channels: Structure, Regulation, and Function
Stephen F. Traynelis,Lonnie P. Wollmuth,Chris J. McBain,Frank S. Menniti,Katie M. Vance,Kevin K. Ogden,Kasper B. Hansen,Hongjie Yuan,Scott J. Myers,Raymond Dingledine +9 more
TL;DR: This review discusses International Union of Basic and Clinical Pharmacology glutamate receptor nomenclature, structure, assembly, accessory subunits, interacting proteins, gene expression and translation, post-translational modifications, agonist and antagonist pharmacology, allosteric modulation, mechanisms of gating and permeation, roles in normal physiological function, as well as the potential therapeutic use of pharmacological agents acting at glutamate receptors.
Related Papers (5)
Neuron-Microglia Interactions in Motor Neuron Degeneration. The Inflammatory Hypothesis in Amyotrophic Lateral Sclerosis Revisited.
Manuel J. Rodriguez,Nicole Mahy +1 more