Motor cortical neuronal hyperexcitability associated with α-synuclein aggregation by Liqiang Chen, Hiba Douja Chehade, Hong-Yuan Chu

“…Specifically, αSyn aggregates-bearing intratelencephalic neurons (ITNs) showed hyperexcitability, increased input resistance, and decreased cell capacitance, which were associated with impaired HCN channel function. …”
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Dysregulation of the Autophagy-Endolysosomal System in Amyotrophic Lateral Sclerosis and Related Motor Neuron Diseases by Asako Otomo, Lei Pan, Shinji Hadano

“…Amyotrophic lateral sclerosis (ALS) is a heterogeneous group of incurable motor neuron diseases (MNDs) characterized by a selective loss of upper and lower motor neurons in the brain and spinal cord. …”
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Identifying key signs of motor neurone disease in primary care: a nested case–control study using the QResearch database by Aleksandar Radunović, Carol Coupland, Julia Hippisley-Cox, Christopher J McDermott, Tom A Ranger, Judith Burchardt, Xue W Mei

“…Objective To confirm the symptoms and signs for motor neuron disease (MND) in the Red Flag tool; to quantify the extent to which the key symptoms and signs are associated with MND; and to identify additional factors which may be helpful within the primary care setting in recognition of possible MND and triggering timely referral to neurology specialists.Design A nested case–control study.Setting 1292 UK general practices contributing to the QResearch primary care database, linked to hospital and mortality data.Participants Baseline cohort included 16.8 million individuals aged 18 years and over without a diagnosis of MND at study entry and with more than 3 years of digitalised information available. …”
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Enhanced Interleukin 6 Trans-Signaling Modulates Disease Process in Amyotrophic Lateral Sclerosis Mouse Models by Carol Milligan, Dale O. Cowley, William Stewart, Alyson M. Curry, Elizabeth Forbes, Brian Rector, Annette Hastie, Liang Liu, Gregory A. Hawkins

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Transfection of the BDNF Gene in the Surviving Dopamine Neurons in Conjunction with Continuous Administration of Pramipexole Restores Normal Motor Behavior in a Bilateral Rat Model... by Alina Benítez-Castañeda, Verónica Anaya-Martínez, Armando de Jesús Espadas-Alvarez, Ana Luisa Gutierrez-Váldez, Luis Fernando Razgado-Hernández, Patricia Emmanuelle Reyna-Velazquez, Liz Quintero-Macias, Daniel Martínez-Fong, Benjamín Florán-Garduño, Jorge Aceves

“…In Parkinson’s disease (PD), progressive degeneration of nigrostriatal innervation leads to atrophy and loss of dendritic spines of striatal medium spiny neurons (MSNs). …”
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KIF5A regulates axonal repair and time-dependent axonal transport of SFPQ granules and mitochondria in human motor neurons by Irune Guerra San Juan, Jessie W. Brunner, Kevin Eggan, Ruud F. Toonen, Matthijs Verhage

“…Mutations in the microtubule-binding motor protein kinesin 5 A (KIF5A) are implicated in several adult-onset motor neuron diseases, including Amyotrophic Lateral Sclerosis, Spastic Paraplegia Type 10 and Charcot-Marie-Tooth Disease Type 2. …”
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Loss of MEF2C function by enhancer mutation leads to neuronal mitochondria dysfunction and motor deficits in mice by Ali Yousefian-Jazi, Suhyun Kim, Jiyeon Chu, Seung-Hye Choi, Phuong Thi Thanh Nguyen, Uiyeol Park, Min-gyeong Kim, Hongik Hwang, Kyungeun Lee, Yeyun Kim, Seung Jae Hyeon, Hyewhon Rhim, Hannah L. Ryu, Grewo Lim, Thor D. Stein, Kayeong Lim, Hoon Ryu, Junghee Lee

“…Conclusions Together, MEF2C dysregulation by the enhancer mutation leads to mitochondrial dysfunction and oxidative stress, which are prevalent features in motor neuronal damage and ALS pathogenesis. This genetic and epigenetic crosstalk mechanism provides insights for advancing our understanding of motor neuron disease and developing effective treatments.…”
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In silico analysis of hub genes and regulatory networks implicates the putamen in non-motor Parkinson’s disease disorders by Stephen Tunmise Akanbi, Joshua Ayodele Yusuf, Thompson Oluwaferanmi Ayandele, Dayo Samson Oladipupo, Darasimi Racheal Olorunlowu, Blessing Dorcas Olawuyi, Joshua Oluwafisayo Adepoju, Dolapo Emmanuel Opasina, Elizabeth Kehinde Opoola, Uchenna Victor Ugwuanyi, Olufunto Omodele Adeleye

“…Abstract Background Parkinson’s disease (PD) is a neurodegenerative condition marked by the gradual degeneration of dopaminergic neurons in the substantia nigra, leading to depletion of the dopaminergic neurons in the substantia nigra as well as degeneration as and decreased activity in the putamen. …”
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Treatment Tactics for Psychosis in Parkinson’s Disease: A Literature Review by Daiva Milmantienė

“…The loss of dopaminergic neurons and disturbances in their connections cause deficient signaling of dopamine circuits, which results in motor and non-motor symptoms. …”
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Substantia nigra alterations in mice modeling Parkinson’s disease by I. N. Rozhkova, S. V. Okotrub, E. Yu. Brusentsev, T. A. Rakhmanova, D. A. Lebedeva, V. S. Kozeneva, N. A. Shavshaeva, N. V. Khotskin, S. Ya. Amstislavsky

“…The aim of the current study was to investigate motor coordination and body balance, as well as dopaminergic neuronal density and alpha-synuclein accumulation in the substantia nigra in male B6.Cg-Tg mice at the age of six months. …”
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The role of CXCL12/CXCR4/CXCR7 axis in cognitive impairment associated with neurodegenerative diseases by Rojin Sarallah, Shima Jahani, Alireza Soltani Khaboushan, Amir Kian Moaveni, Maryam Amiri, Masoumeh Majidi Zolbin

“…ALS research indicates that the CXCL12/CXCR4/CXCR7 pathway is involved in motor neuron degeneration and associated cognitive deficits. …”
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Endothelial and neuronal engagement by AAV-BR1 gene therapy alleviates neurological symptoms and lipid deposition in a mouse model of Niemann-Pick type C2 by Charlotte Laurfelt Munch Rasmussen, Signe Frost Frederiksen, Christian Würtz Heegaard, Maj Schneider Thomsen, Eva Hede, Bartosz Laczek, Jakob Körbelin, Daniel Wüstner, Louiza Bohn Thomsen, Markus Schwaninger, Ole N. Jensen, Torben Moos, Annette Burkhart

“…We hypothesized that an adeno-associated virus (AAV-BR1) encoding the Npc2 gene could cure neurological symptoms in Npc2−/− mice through transduction of BECs, and possibly neurons via viral passage across the BBB. …”
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Pathogenesis of Disorders of the Motor Function of the Large Intestine in Functional Constipation by M. M. Galagudza, Yu. P. Uspensky, Yu. A. Fominykh, D. Yu. Butko

“…A key role in the FC development belongs to a decrease in the pool of interstitial cells, which play the role of an intestinal pacemaker, due to slowing of their self-renewal. FC-associated changes in the enteric nervous system include a relative excess of the contribution of inhibitory influences and a decrease in the activity of cholinergic and serotonergic neurons that stimulate intestinal motility. …”
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7,8-Dihydroxyflavone Protects Nigrostriatal Dopaminergic Neurons from Rotenone-Induced Neurotoxicity in Rodents by Shuke Nie, Kai Ma, Mingkuan Sun, Matthew Lee, Yang Tan, Guiqin Chen, Zhentao Zhang, Zhaohui Zhang, Xuebing Cao

“…7,8-Dihydroxyflavone (7,8-DHF) is thought to be a promising therapeutic agent for various neurodegenerative diseases. The major purpose of this study was to investigate the neuroprotective effects of 7,8-DHF on the rotenone-induced motor deficit of Parkinson’s disease. …”
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STRUCTURAL AND DYNAMIC PROPERTIES OF MUTANTS OF THE SOD1 PROTEIN ASSOCIATED WITH AMYOTROPHIC LATERAL SCLEROSIS by N. A. Alemasov, N. V. Ivanisenko, V. A. Ivanisenko

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Diagnostics and Treatment of Esophageal Achalasia (Clinical Guidelines of the Russian Gastroenterological Association, Russian Scientific Medical Society of Internal Medicine, Russ... by V. T. Ivashkin, A. S. Trukhmanov, I. V. Maev, O. M. Drapkina, A. I. Martynov, O. A. Storonova, E. A. Godgello, M. P. Korolev, T. L. Lapina, P. V. Pavlov, A. V. Paraskevova, I. A. Tarasova, E. D. Fedorov, A. T. Tskhovrebov, M. P. Shapka, A. L. Shestakov, A. V. Yurasov

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The Role of the Gastrointestinal Microbiota in Parkinson’s Disease by Maurizio Gabrielli, Lorenzo Zileri Dal Verme, Maria Assunta Zocco, Enrico Celestino Nista, Veronica Ojetti, Antonio Gasbarrini

“…Background/Objectives: Parkinson’s disease (PD) is a progressive neurodegenerative disorder characterized by the loss of dopaminergic neurons leading to debilitating motor and non-motor symptoms. …”
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Neurophysiology of Drosophila Models of Parkinson’s Disease by Ryan J. H. West, Rebecca Furmston, Charles A. C. Williams, Christopher J. H. Elliott

“…We provide an insight into the role Drosophila has played in elucidating neurophysiological perturbations associated with Parkinson’s disease- (PD-) related genes. …”
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Predictive Clinical-Biological Markers Over the First 3 Years of Nusinersen Treatment in SMA Type 1 Patients by Mihaela Badina, Andrada Mirea, Corina Sporea, Gabriel Cristian Bejan

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Evolution of Functional and Paraclinical Markers as Predictive Factors in Pediatric Late-Onset SMA Under Nusinersen Treatment: The Role of CSF pNF-H by Mihaela Badina, Gabriel Cristian Bejan, Corina Sporea, Andrada Mirea

“…We assessed pNF-H levels in CSF and serum—neuronal proteins associated with neurodegeneration—as well as serum creatinine levels, a marker of muscle activity, and motor skill scores to evaluate pNF-H’s potential as a predictor of motor development. …”
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