Gogia, Neha Sarkar, Ankita Mehta, Abijeet Singh Ramesh, Nandini Deshpande, Prajakta Kango-Singh, Madhuri Pandey, Udai Bhan Singh, Amit
Published in
Neurobiology of disease
Amyotrophic Lateral Sclerosis (ALS), a late-onset neurodegenerative disorder characterized by the loss of motor neurons in the central nervous system, has no known cure to-date. Disease causing mutations in human Fused in Sarcoma (FUS) leads to aggressive and juvenile onset of ALS. FUS is a well-conserved protein across different species, which pla...
Barrio, Tomás Filali, Hicham Otero, Alicia Sheleby-Elías, Jessica Marín, Belén Vidal, Enric Béringue, Vincent Torres, Juan María Groschup, Martin Andréoletti, Olivier
...
Published in
Scientific Reports
Phenotypic variability in prion diseases, such as scrapie, is associated to the existence of prion strains, which are different pathogenic prion protein (PrPSc) conformations with distinct pathobiological properties. To faithfully study scrapie strain variability in natural sheep isolates, transgenic mice expressing sheep cellular prion protein (Pr...
La Morgia, Chiara Maresca, Alessandra Amore, Giulia Gramegna, Laura Ludovica Carbonelli, Michele Scimonelli, Emanuela Danese, Alberto Patergnani, Simone Caporali, Leonardo Tagliavini, Francesca
...
Published in
Scientific Reports
Wolfram syndrome (WS) is a recessive multisystem disorder defined by the association of diabetes mellitus and optic atrophy, reminiscent of mitochondrial diseases. The role played by mitochondria remains elusive, with contradictory results on the occurrence of mitochondrial dysfunction. We evaluated 13 recessive WS patients by deep clinical phenoty...
Wójtowicz, Sylwia Strosznajder, Anna K. Jeżyna, Mieszko Strosznajder, Joanna B.
Published in
Neurochemical Research
Peroxisome proliferator activated receptor alpha (PPAR-α) belongs to the family of ligand-regulated nuclear receptors (PPARs). These receptors after heterodimerization with retinoid X receptor (RXR) bind in promotor of target genes to PPAR response elements (PPREs) and act as a potent transcription factors. PPAR-α and other receptors from this fami...
Zak, A Siwinska, N Elzinga, S Barker, V D Stefaniak, T Schanbacher, B J Place, N J Niedzwiedz, A Adams, A A
Published in
Domestic animal endocrinology
Age, neurodegenerative disorders, and dysfunction of insulin secretion may be correlated with increased systemic concentrations of acute phase markers. Thus, the study aimed to determine the effect of age, pituitary pars intermedia dysfunction (PPID), and insulin dysregulation (ID) associated with PPID, on markers of the acute phase reaction. Twent...
Hijaz, Baraa A. Volpicelli-Daley, Laura A.
Published in
Molecular Neurodegeneration
The two main pathological hallmarks of Parkinson’s disease are loss of dopamine neurons in the substantia nigra pars compacta and proteinaceous amyloid fibrils composed mostly of α-synuclein, called Lewy pathology. Levodopa to enhance dopaminergic transmission remains one of the most effective treatment for alleviating the motor symptoms of Parkins...
Cirrincione, Anthony M. Pellegrini, Adriana D. Dominy, Jessica R. Benjamin, Marisa E. Utkina-Sosunova, Irina Lotti, Francesco Jergova, Stanislava Sagen, Jacqueline Rieger, Sandra
Published in
Scientific Reports
Paclitaxel induces peripheral neuropathy as a side effect of cancer treatment. The underlying causes are unclear, but epidermal, unmyelinated axons have been shown to be the first to degenerate. We previously utilized an in vivo zebrafish model to show that the epidermal matrix-metalloproteinase 13 (MMP-13) induces degeneration of unmyelinated axon...
Nakano, Yuki Hirooka, Kazuyuki Chiba, Yoichi Ueno, Masaki Ojima, Daiki Hossain, Md Razib Takahashi, Hiroo Yamamoto, Tohru Kiuchi, Yoshiaki
Published in
Cell Death & Disease
Maintenance of retinal ganglion cells (RGCs) activity is relied on axonal transport conveying materials required for their survival such as neurotrophic factors. Kinesin-1 undergoes anterograde transport in axons, and Alcadein α (Alcα; also called calsyntenin-1) is a major cargo adaptor protein that can drive kinesin-1 to transport vesicles contain...
Varga, Júlia Dér, Nikolett Petra Zsindely, Nóra Bodai, László
Published in
Nutritional neuroscience
Objectives: Green tea infusion contains a complex mixture of polyphenolic compounds that were shown to provide health benefits. It was previously demonstrated that (-)-epigallocatechin-3-gallate, one of the major polyphenols present in green tea, has a suppressing effect on various aspects of pathogenesis in models of Huntington's disease (HD), an ...
Yan, Xu Uronen, Riikka-Liisa Huttunen, Henri J
Published in
Seminars in cell & developmental biology
α-synuclein and Tau are proteins prone to pathological misfolding and aggregation that are normally found in the presynaptic and axonal compartments of neurons. Misfolding initiates a homo-oligomerization and aggregation cascade culminating in cerebral accumulation of aggregated α-synuclein and Tau in insoluble protein inclusions in multiple neurod...
