نتایج جستجو برای: associated neurodegeneration

تعداد نتایج: 1531207  

پایان نامه :دانشگاه آزاد اسلامی - دانشگاه آزاد اسلامی واحد علوم دارویی - پژوهشکده علوم 1392

purpose a metabolic abnormality such as obesity is a major obstacle in the maintenance of the human health system and causes various chronic diseases including type 2 diabetes, hypertension, cardiovascular diseases, as well as various cancers. this study was designed to summarize the recent scientific knowledge regarding the anti-obesity role of curcumin (diferuloylmethane), which is isolated f...

Journal: :FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2004
Gwenn A Garden Weiqun Guo Suman Jayadev Christina Tun Stephanie Balcaitis Jo Choi Thomas J Montine Thomas Möller Richard S Morrison

HIV infection of the central nervous system leads to HIV-associated dementia (HAD) in a substantial subset of infected individuals. The pathogenesis of neuronal dysfunction in HAD is not well understood, but previous studies have demonstrated evidence for activation of apoptotic pathways. The tumor suppressor transcription factor p53 is an apical mediator of neuronal apoptosis following a varie...

Journal: :Cell 2010
Krzysztof Rogowski Juliette van Dijk Maria M. Magiera Christophe Bosc Jean-Christophe Deloulme Anouk Bosson Leticia Peris Nicholas D. Gold Benjamin Lacroix Montserrat Bosch Grau Nicole Bec Christian Larroque Solange Desagher Max Holzer Annie Andrieux Marie-Jo Moutin Carsten Janke

Polyglutamylation is a posttranslational modification that generates glutamate side chains on tubulins and other proteins. Although this modification has been shown to be reversible, little is known about the enzymes catalyzing deglutamylation. Here we describe the enzymatic mechanism of protein deglutamylation by members of the cytosolic carboxypeptidase (CCP) family. Three enzymes (CCP1, CCP4...

Journal: :The Journal of clinical investigation 2013
Xing Guo Marie-Helene Disatnik Marie Monbureau Mehrdad Shamloo Daria Mochly-Rosen Xin Qi

Huntington's disease (HD) is the result of expression of a mutated Huntingtin protein (mtHtt), and is associated with a variety of cellular dysfunctions including excessive mitochondrial fission. Here, we tested whether inhibition of excessive mitochondrial fission prevents mtHtt-induced pathology. We developed a selective inhibitor (P110-TAT) of the mitochondrial fission protein dynamin-relate...

Journal: :Biochemical Society symposium 1999
S J Tabrizi A H Schapira

The central nervous system has a particularly high energy requirement, thus making it very susceptible to defects in mitochondrial function. A number of neurodegenerative diseases, in particular Parkinson's disease (PD), Huntington's disease (HD) and Friedreich's ataxia (FRDA), are associated with mitochondrial dysfunction. The identification of a mitochondrial complex-I defect in PD provides a...

Journal: :EMBO molecular medicine 2016
Dario Brunetti Janniche Torsvik Cristina Dallabona Pedro Teixeira Pawel Sztromwasser Erika Fernandez-Vizarra Raffaele Cerutti Aurelio Reyes Carmela Preziuso Giulia D'Amati Enrico Baruffini Paola Goffrini Carlo Viscomi Ileana Ferrero Helge Boman Wenche Telstad Stefan Johansson Elzbieta Glaser Per M Knappskog Massimo Zeviani Laurence A Bindoff

Mitochondrial dysfunction and altered proteostasis are central features of neurodegenerative diseases. The pitrilysin metallopeptidase 1 (PITRM1) is a mitochondrial matrix enzyme, which digests oligopeptides, including the mitochondrial targeting sequences that are cleaved from proteins imported across the inner mitochondrial membrane and the mitochondrial fraction of amyloid beta (Aβ). We iden...

Journal: :Archives of neurology 2004
Thomas Müller Kathrin Renger Wilfried Kuhn

BACKGROUND Levodopa metabolism via catechol O-methyltransferase increases levels of the neurotoxin homocysteine, which induces an axonal-accentuated degeneration in sensory peripheral nerves in vitro. OBJECTIVES To demonstrate associations among daily levodopa/dopa decarboxylase inhibitor intake, total homocysteine plasma (tHcy) levels, and electrophysiologic sural nerve conduction findings. ...

Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2002
Gwenn A Garden Samantha L Budd Elena Tsai Lisa Hanson Marcus Kaul Danielle M D'Emilia Robert M Friedlander Junying Yuan Eliezer Masliah Stuart A Lipton

Many patients infected with human immunodeficiency virus-1 (HIV-1) develop a syndrome of neurologic deterioration known as HIV-associated dementia (HAD). Neurons are not productively infected by HIV-1; thus, the mechanism of HIV-induced neuronal injury remains incompletely understood. Several investigators have observed evidence of neuronal injury, including dendritic degeneration, and apoptosi...

2013
A Li R Paudel R Johnson R Courtney A J Lees J L Holton J Hardy T Revesz H Houlden

AIMS Mutations in the pantothenate kinase 2 gene (PANK2) are responsible for the most common type of neurodegeneration with brain iron accumulation (NBIA), known as pantothenate kinase-associated neurodegeneration (PKAN). Historically, NBIA is considered a synucleinopathy with numerous reports of NBIA cases with Lewy bodies and Lewy neurites and some cases reporting additional abnormal tau accu...

Journal: :Neurology 2005
M T Pellecchia E M Valente L Cif S Salvi A Albanese V Scarano U Bonuccelli A R Bentivoglio A D'Amico C Marelli A Di Giorgio P Coubes P Barone B Dallapiccola

Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal-recessive disorder caused by mutations in the PANK2 gene. The authors report clinical and genetic findings of 16 patients with PKAN. The authors identified 12 mutations in the PANK2 gene, five of which were new. Only nine patients could be classified as classic or atypical PKAN, and intermediate phenotypes are described...

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