Results 41 to 50 of about 145,378 (214)

β-microglobulin amyloid fibril-induced membrane disruption is enhanced by endosomal lipids and acidic pH [PDF]

open access: yes, 2014
Although the molecular mechanisms underlying the pathology of amyloidoses are not well understood, the interaction between amyloid proteins and cell membranes is thought to play a role in several amyloid diseases. Amyloid fibrils of β-microglobulin (βm),
Eric W. Hewitt   +49 more
core   +1 more source

Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases

open access: yesFrontiers in Cellular Neuroscience, 2019
Histopathological studies revealed that progressive neuropathies including Alzheimer, and Prion diseases among others, include accumulations of misfolded proteins intracellularly, extracellularly, or both.
Emiliano Zamponi, Gustavo F. Pigino
doaj   +1 more source

Cerebral amyloid angiopathy : new insights from transgenic mice [PDF]

open access: yes, 2004
Cerebral amyloid angiopathy (CAA) is characterized by the deposition of congophilic material within the walls of small to medium-sized blood vessels of the brain and leptomeninges.
Herzig, Martin C.
core   +1 more source

Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs   +7 more
wiley   +1 more source

Mitochondria‐Targeted Nanotherapies in Aging Neurodegenerative Disorders: Emerging Prospects and Clinical Potential

open access: yesAdvanced Healthcare Materials, EarlyView.
Mitochondria‐targeted nanotherapies emerge as a promising strategy for combating aging‐associated neurodegenerative disorders (NDs) by restoring mitochondrial function, reducing oxidative stress, and improving neuronal survival. Recent advances in nanotechnology, therapeutic delivery, and translational research are highlighted, providing insights into ...
Dnyandev G. Gadhave   +8 more
wiley   +1 more source

Ectodomain shedding of the amyloid precursor protein: Cellular control mechanisms and novel modifiers [PDF]

open access: yes, 2006
Proteolytic cleavage in the ectodomain of the amyloid precursor protein (APP) is a key regulatory step in the generation of the Alzheimer's disease amyloid-beta (A beta) pepticle and occurs through two different protease activities termed alpha- and beta-
Lichtenthaler, Stefan F.
core   +1 more source

Distribution of Big Tau Isoforms in the Human Central and Peripheral Nervous System

open access: yesAnnals of Neurology, EarlyView.
Objective Tau is widely studied in neurodegeneration, yet most work has focused on canonical brain tau isoforms. A longer isoform, “big tau,” produced by inclusion of exon 4a, is expressed in the peripheral nervous system (PNS) and central nervous system (CNS) regions.
Rama Krishna Koppisetti   +17 more
wiley   +1 more source

Structure of the human TRiC/CCT Subunit 5 associated with hereditary sensory neuropathy

open access: yesScientific Reports, 2017
The human chaperonin TRiC consists of eight non-identical subunits, and its protein-folding activity is critical for cellular health. Misfolded proteins are associated with many human diseases, such as amyloid diseases, cancer, and neuropathies, making ...
Jose H. Pereira   +5 more
doaj   +1 more source

Fingolimod attenuates experimental autoimmune neuritis and contributes to Schwann cell-mediated axonal protection

open access: yesJournal of Neuroinflammation, 2017
Background Fingolimod, a sphingosine-1-phosphate receptor modulator with well-described immunomodulatory properties involving peripheral immune cell trafficking, was the first oral agent approved for the treatment of relapsing remitting multiple ...
Björn Ambrosius   +5 more
doaj   +1 more source

Amyloid and tau in the brain in sporadic Alzheimer’s disease: defining the chicken and the egg [PDF]

open access: yes, 2014
In the October 2013 issue of Acta Neuropathologica there were three very interesting articles on: Amyloid or tau: the chicken or the egg? In the first article, David Mann and John Hardy argued that the deposition of aggregated amyloid β (Aβ) protein in ...
Cheryl A. Hawkes   +5 more
core   +1 more source

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