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| 1 | Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure显示文摘The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer's disease, Huntington's disease, Parkinson's disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha(PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas(hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival. | Jessica D.Panes Aline Wendt Oscar Ramirez-Molina Patricio A.Castro Jorge Fuentealba | 2022 | Neural Regeneration Research2022，17，2： | 6 |
| 2 | Neuroprotective effects of erythropoietin on neurodegenerative and ischemic brain diseases: the role of erythropoietin receptor显示文摘Erythropoietin(Epo) is a fundamental hormone in the regulation of hematopoiesis, and other secondary roles mediated by the binding of the hormone to its specific receptor(Epo R), which leads to an activation of key signaling pathways that induce an increase in cell differentiation, apoptosis control and neuroprotection. It has been suggested that their function depends on final conformation of glycosylations, related with affinity to the receptor and its half-life. The presence of Epo R has been reported in different tissues including central nervous system, where it has been demonstrated to exert a neuroprotective function against oxidative stress conditions, such as ischemic injury and neurodegenerative diseases. There is also evidence of an increase in Epo R expression in brain cell lysates of Alzheimer's patients with respect to healthy patients. These results are related with extensive in vitro experimental data of neuroprotection obtained from cell lines, primary cell cultures and hippocampal slices. Additionally, this data is correlated with in vivo experiments(water maze test) in mouse models of Alzheimer's disease where Epo treatment improved cognitive function. These studies support the idea that receptor activation induces a neuroprotective effect in neurodegenerative disorders including dementias, and especially Alzheimer's disease. Taken together, available evidence suggests that Epo appears to be a central element for Epo R activation and neuroprotective properties in the central nervous system. In this review, we will describe the mechanisms associated with neuroprotection and its relation with the activation of Epo R in order with identify new targets to develop pharmacological strategies. | Carolina Castillo Hernández Carlos Felipe Burgos Angela Hidalgo Gajardo Tiare Silva-Grecchi Javiera Gavilan Jorge Roberto Toledo Jorge Fuentealba | 2017 | Neural Regeneration Research2017，12，9： | 5 |
| 3 | Factor analysis identifies subgroups of constipation显示文摘AIM:To determine whether distinct symptom groupings exist in a constipated population and whether such grouping might correlate with quantifiable pathophysiological measures of colonic dysfunction.METHODS:One hundred and ninety-one patients presenting to a Gastroenterology clinic with constipation and 32 constipated patients responding to a newspaper advertisement completed a 53-item,wide-ranging selfreport questionnaire.One hundred of these patients had colonic transit measured scintigraphically.Factor analysis determined whether constipation-related symptoms grouped into distinct aspects of symptomatology.Cluster analysis was used to determine whether indi-vidual patients naturally group into distinct subtypes.RESULTS:Cluster analysis yielded a 4 cluster solution with the presence or absence of pain and laxative unresponsiveness providing the main descriptors.Amongst all clusters there was a considerable proportion of patients with demonstrable delayed colon transit,irritable bowel syndrome positive criteria and regular stool frequency.The majority of patients with these characteristics also reported regular laxative use.CONCLUSION:Factor analysis identified four constipation subgroups,based on severity and laxative unresponsiveness,in a constipated population.However,clear stratification into clinically identifiable groups remains imprecise. | Philip G Dinning Mike Jones Linda Hunt Sergio E Fuentealba Jamshid Kalanter Denis W King David Z Lubowski Nicholas J Talley Ian J Cook | 2011 | World Journal of Gastroenterology2011，17，11： | 3 |
| 4 | Aromaticity and electronic structure of silabenzenes, possible existence of a new cluster Si6 Li6 显示文摘 | Santos J C Fuentealba P | 2007 | Chem Phys Lett2007，443，456： | 1 |
| 5 | Animal models of copper-associated liver disease显示文摘 | Fuentealba IC Aburto EM | 2003 | Comp Hepatol2003，2，1： | 1 |
| 6 | Extracellular matrix histone H1 binds to Perlecan, is present in regenerating skeletal muscle and stimulates myoblast proliferation 显示文摘 | Henriquez JP Casar JC Fuentealba L | 2002 | Cell Sci2002，115，10： | 1 |
| 7 | Enteric infection with an obligate intracellular parasite, Encephalitozoon cunicull,in an experimental model显示文摘 | Wicher V Baughn R E Fuentealba I C | 1991 | Infect Immun1991，59，2： | 1 |
| 8 | Theoretical framework for assessment of risks posed to Canadian forests by invasive insect species显示文摘 | Fuentealba A Alfaro R Bauce | 2013 | Forest Ecology and Management2013，302，： | 1 |
| 9 | Experimental infection and horizontal transmissiom of Piscirickettsia salmonis in freshwater-raised Atlantic salmon, Salmo salar L显示文摘 | Almendras F E Fuentealba I C Jones S R M | 1997 | J Fish Dis1997，20，： | 1 |
| 10 | Adult Neural Stem Cells Bridge Their Niche显示文摘 | Luis C. Fuentealba Kirsten Obernier Arturo Alvarez-Buylla | 2012 | Cell Stem Cell2012，，6： | 1 |
| 11 | Expression of the hemagglutinin HA1 subunit of the equine influenza virus using a baculovirus expression system 显示文摘 | Sguazza GH Fuentealba NA Tizzano MA et l | 2013 | Rev Argent Microbiol2013，45，4： | 1 |
| 12 | Electronic structure and bonding of the ground state of alkaline-earth-metal monoxides and carbides显示文摘 | Fuentealba P Savin A | 2000 | J Phys Chem A2000，104，10： | 1 |
| 13 | Animal models of copper-associated liverdisease显示文摘 | Fuentealba C Aburto EM | 2003 | Comp Hepatol2003，2，1： | 1 |
| 14 | Morphological and biochemical aasessment of the liver response to excess dieuiry copper in Fischer 344 rats显示文摘 | ABURTO EM CRIBB AE FUENTEALBA IC | 2001 | Can J Vet Res2001，65，： | 1 |
| 15 | Theoretical study of the interaction of molecular oxygen with copper clusters 显示文摘 | Florez E Tiznado W Mondrag6n F Fuentealba P | 2005 | JPhys Chem A2005，109，： | 1 |
| 16 | Signal transduction during amyloid-beta-peptide neurotoxcity : role in Alzheimer disease 显示文摘 | Fuentealba RA Farias G Sclaeu J | 2004 | Brain Res Rev2004，47，13： | 1 |
| 17 | A proper account of corepolarization with pseudopotentials:Single valence-electron alkali compounds显示文摘 | Fuentealba P Preuss H Szentpaly L V | 1982 | Chem Phys Lett1982，89，： | 1 |
| 18 | Aggregation behavior of pegylated bile acid derivatives显示文摘 | LE DEVEDEC F FUENTEALBA D STRAND- MAN S | 2012 | Langmuir2012，28，13： | 1 |
| 19 | Synthesis, spectral,structural, second-order nonlinear opticalproperties and theoretical studies on new organometallic donor-acceptor substituted nickeK H ) and copper( fl ) unsymmetricalSchiff-base complexes显示文摘 | TRUJILLO A FUENTEALBA M CARRILLO D | 2010 | Inorg Chem2010，49，： | 1 |
| 20 | Expression of COUP-TFII nuclear receptor in restricted GABAergic neuronal pop- ulations in the adult rat hippocampus 显示文摘 | Fuentealba P Klausberger T Karayannis T | 2010 | J Neurosci2010，30，： | 1 |
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