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https://www.readbyqxmd.com/read/28934388/loss-of-slc25a46-causes-neurodegeneration-by-affecting-mitochondrial-dynamics-and-energy-production-in-mice
#1
Zhuo Li, Yanyan Peng, Robert B Hufnagel, Yueh-Chiang Hu, Chuntao Zhao, Luis F Queme, Zaza Khuchua, Ashley M Driver, Fei Dong, Q Richard Lu, Diana M Lindquist, Michael P Jankowski, Rolf W Stottmann, Winston W Y Kao, Taosheng Huang
Recently, we identified biallelic mutations of SLC25A46 in patients with multiple neuropathies. Functional studies revealed that SLC25A46 may play an important role in mitochondrial dynamics by mediating mitochondrial fission. However, the cellular basis and pathogenic mechanism of the SLC25A46-related neuropathies are not fully understood. Thus, we generated a Slc25a46 knock-out mouse model. Mice lacking SLC25A46 displayed severe ataxia, mainly caused by degeneration of Purkinje cells. Increased numbers of small, unmyelinated and degenerated optic nerves as well as loss of retinal ganglion cells indicated optic atrophy...
October 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28931570/mitochondrial-dna-double-strand-breaks-in-oligodendrocytes-cause-demyelination-axonal-injury-and-cns-inflammation
#2
Pernille M Madsen, Milena Pinto, Shreyans Patel, Stephanie McCarthy, Han Gao, Mehran Taherian, Shaffiat Karmally, Claudia V Pereira, Galina Dvoriantchikova, Dmitry Ivanov, Kenji F Tanaka, Carlos T Moraes, Roberta Brambilla
Mitochondrial dysfunction has been implicated in the pathophysiology of neurodegenerative disorders, including multiple sclerosis (MS). To date, the investigation of mitochondrial dysfunction in MS has focused exclusively on neurons, with no studies exploring whether dysregulation of mitochondrial bioenergetics and/or genetics in oligodendrocytes might be associated with the etiopathogenesis of MS and other demyelinating syndromes. To address this question, we established a mouse model where mitochondrial DNA (mtDNA) double-strand breaks (DSB) were specifically induced in myelinating oligodendrocytes (PLP:mtPstI mice) by expressing a mitochondrial-targeted endonuclease, mtPstI, starting at 3 weeks of age...
September 20, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28930688/the-heterochronic-gene-lin-14-controls-axonal-degeneration-in-c-%C3%A2-elegans-neurons
#3
Fiona K Ritchie, Rhianna Knable, Justin Chaplin, Rhiannon Gursanscky, Maria Gallegos, Brent Neumann, Massimo A Hilliard
The disproportionate length of an axon makes its structural and functional maintenance a major task for a neuron. The heterochronic gene lin-14 has previously been implicated in regulating the timing of key developmental events in the nematode C. elegans. Here, we report that LIN-14 is critical for maintaining neuronal integrity. Animals lacking lin-14 display axonal degeneration and guidance errors in both sensory and motor neurons. We demonstrate that LIN-14 functions both cell autonomously within the neuron and non-cell autonomously in the surrounding tissue, and we show that interaction between the axon and its surrounding tissue is essential for the preservation of axonal structure...
September 19, 2017: Cell Reports
https://www.readbyqxmd.com/read/28929513/association-between-hba1c-and-peripheral-neuropathy-in-a-10-year-follow-up-study-of-people-with-normal-glucose-tolerance-impaired-glucose-tolerance-and-type-2-diabetes
#4
M Peterson, R Pingel, N Lagali, L B Dahlin, O Rolandsson
AIMS: To explore the association between HbA1c and sural nerve function in a group of people with normal glucose tolerance, impaired glucose tolerance or Type 2 diabetes. METHODS: We conducted a 10-year follow-up study in 87 out of an original 119 participants. At study commencement (2004), 64 men and 55 women (mean age 61.1 years) with normal glucose tolerance (n=39), impaired glucose tolerance (n=29), or Type 2 diabetes (n=51) were enrolled. At the 2014 follow-up (men, n=46, women, n=41; mean age 71...
September 20, 2017: Diabetic Medicine: a Journal of the British Diabetic Association
https://www.readbyqxmd.com/read/28928628/axonal-degeneration-during-aging-and-its-functional-role-in-neurodegenerative-disorders
#5
REVIEW
Natalia Salvadores, Mario Sanhueza, Patricio Manque, Felipe A Court
Aging constitutes the main risk factor for the development of neurodegenerative diseases. This represents a major health issue worldwide that is only expected to escalate due to the ever-increasing life expectancy of the population. Interestingly, axonal degeneration, which occurs at early stages of neurodegenerative disorders (ND) such as Alzheimer's disease, Amyotrophic lateral sclerosis, and Parkinson's disease, also takes place as a consequence of normal aging. Moreover, the alteration of several cellular processes such as proteostasis, response to cellular stress and mitochondrial homeostasis, which have been described to occur in the aging brain, can also contribute to axonal pathology...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28927011/assessing-autophagy-in-sciatic-nerves-of-a-rat-model-that-develops-inflammatory-autoimmune-peripheral-neuropathies
#6
REVIEW
Susana Brun, Nicolas Schall, Hélène Jeltsch-David, Jérôme de Sèze, Sylviane Muller
The rat sciatic nerve has attracted widespread attention as an excellent model system for studying autophagy alterations in peripheral neuropathies. In our laboratory, we have developed an original rat model, which we used currently in routine novel drug screening and to evaluate treatment strategies for chronic inflammatory demyelinating polyneuropathy (CIDP) and other closely related diseases. Lewis rats injected with the S-palmitoylated P0(180-199) peptide develop a chronic, sometimes relapsing-remitting type of disease...
September 18, 2017: Cells
https://www.readbyqxmd.com/read/28926781/a-leukomyeloencephalopathy-of-unknown-origin-in-an-azawakh-dog
#7
Maria Teresa Mandara, Alice Reginato, Federica Balducci, Marco Bernardini
A diffuse bilaterally symmetrical leukomyeloencephalopathy was observed in a 6-year-old male Azawakh dog showing a slowly progressive ataxia of six months duration associated with sensory disorders. Severe bilaterally symmetrical demyelination and vacuolisation were confined to the dorsal columns along the entire spinal cord with a minor axonal degeneration. The main changes of myelin sheaths consisted in splitting and intramyelin vacuolization. Naked axons were scattered in a network of astrocytic processes and collagen fibres...
September 5, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/28926646/vascular-hypoperfusion-in-acute-optic-neuritis-is-a-potentially-new-neurovascular-model-for-demyelinating-diseases
#8
Ta-Ching Chen, Chao-Yuan Yeh, Chao-Wen Lin, Chung-May Yang, Chang-Hao Yang, I-Hung Lin, Pao-Yang Chen, Jung-Yu Cheng, Fung-Rong Hu
PURPOSE: Optic neuritis is highly correlated with multiple sclerosis and is a major cause of acute visual loss and long-term neuronal degeneration. Primary cerebral hypoperfusion has been reported in brain demyelinating diseases. This study investigated whether peripapillary perfusion is changed in patients with acute optic neuritis (AON). METHODS: This three-year cohort study was conducted from September 1 2012, to August 31, 2015. Two hundred and forty-one patients with non-glaucomatous acute optic neuropathy were screened, and 42 non-highly myopic patients who had suffered their first episode of unilaterally idiopathic AON were studied...
2017: PloS One
https://www.readbyqxmd.com/read/28919237/inflammatory-responses-in-multiple-sclerosis-normal-appearing-white-matter-and-in-non-immune-mediated-neurological-conditions-with-wallerian-axonal-degeneration-a-comparative-study
#9
M Vercellino, C Trebini, E Capello, G L Mancardi, M T Giordana, P Cavalla
Inflammatory-like changes in the white matter (WM) are commonly observed in conditions of axonal degeneration by different etiologies. This study is a systematic comparison of the principal features of the inflammatory-like changes in the WM in different pathological conditions characterized by axonal damage/degeneration, focusing in particular on Multiple Sclerosis (MS) normal-appearing white matter (NAWM) compared to non immune-mediated disorders. The study was performed on sections of NAWM from 15 MS cases, 11 cases of non immune-mediated disorders with wallerian axonal degeneration (stroke, trauma, amyotrophic lateral sclerosis), 3 cases of viral encephalitis, 6 control cases...
September 8, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28912674/a-mechanistic-understanding-of-axon-degeneration-in-chemotherapy-induced-peripheral-neuropathy
#10
REVIEW
Yusuke Fukuda, Yihang Li, Rosalind A Segal
Chemotherapeutic agents cause many short and long term toxic side effects to peripheral nervous system (PNS) that drastically alter quality of life. Chemotherapy-induced peripheral neuropathy (CIPN) is a common and enduring disorder caused by several anti-neoplastic agents. CIPN typically presents with neuropathic pain, numbness of distal extremities, and/or oversensitivity to thermal or mechanical stimuli. This adverse side effect often requires a reduction in chemotherapy dosage or even discontinuation of treatment...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28912156/neutrophils-are-critical-for-myelin-removal-in-a-peripheral-nerve-injury-model-of-wallerian-degeneration
#11
Jane A Lindborg, Matthias Mack, Richard E Zigmond
Wallerian degeneration (WD) is considered an essential preparatory stage to the process of axonal regeneration. In the peripheral nervous system, infiltrating monocyte-derived macrophages that utilize the chemokine receptor CCR2 to gain entry to injured tissues from the bloodstream, are purportedly necessary for efficient WD. However, our lab has previously reported that myelin clearance in the injured sciatic nerve proceeds unhindered in the Ccr2(-/-) mouse model. Here, we extensively characterize WD in male Ccr2(-/-) mice and identify a compensatory mechanism of WD that is facilitated primarily by neutrophils...
September 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28911883/tnf-superfamily-member-april-enhances-midbrain-dopaminergic-axon-growth-and-contributes-to-the-nigrostriatal-projection-in-vivo
#12
Thomas G McWilliams, Laura Howard, Sean Wyatt, Alun M Davies
We have studied the role of the tumor necrosis factor superfamily member APRIL in the development of embryonic mouse midbrain dopaminergic neurons in vitro and in vivo. In culture, soluble APRIL enhanced axon growth during a window of development between E12 and E14 when nigrostriatal axons are growing to their targets in the striatum in vivo. April transcripts were detected in both the striatum and midbrain during this period and at later stages. The axon growth-enhancing effect of APRIL was similar to that of glial cell-derived neurotrophic factor (GDNF), but in contrast to GDNF, APRIL did not promote the survival of midbrain dopaminergic neurons...
September 11, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28904462/clinical-spectrum-therapeutic-outcomes-and-prognostic-predictors-in-sjogren-s-syndrome-associated-neuropathy
#13
Ajith Sivadasan, Karthik Muthusamy, Bimal Patel, Rohit Ninan Benjamin, A T Prabhakar, Vivek Mathew, Sanjith Aaron, Mathew Alexander
OBJECTIVES: There are limited data regarding long-term follow-up and therapeutic outcomes in Sjogren's syndrome (SS)-associated peripheral neuropathy. In this study, we aim to study the clinical, electrophysiological spectrum and therapeutic responses among the different subtypes of SS-associated neuropathy. The predictors of suboptimal treatment response will be identified. METHODS: The study included a retrospective cohort of patients with SS-associated neuropathy between January 2012 and November 2015...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28901548/primary-congenital-neuroaxonal-dystrophy-with-peripheral-nerve-demyelination-in-merino-border-leicester-%C3%A3-polled-dorset-lambs
#14
M C Hawes, J W Finnie, I V Jerrett, R Badman, M Scott
CASE REPORT: Clinicopathological features of neuroaxonal dystrophy (NAD) in newborn, Merino-Border Leicester × Polled Dorset lambs are described. The affected lambs were unable to walk at birth and microscopic examination of brainstem and spinal cord sections revealed bilaterally symmetrical accumulations of axonal swellings (spheroids), the histological hallmark of primary NAD. The neurological deficit was also exacerbated by myelin loss and secondary axonal degeneration, particularly in the spinal cord and sciatic nerves, but also, to a more limited extent, in brainstem and spinal nerves...
September 13, 2017: Australian Veterinary Journal
https://www.readbyqxmd.com/read/28899595/peripheral-nerve-pathology-at-fixed-stage-in-spinal-muscular-atrophy-with-respiratory-distress-type-1
#15
Azusa Ikeda, Sumimasa Yamashita, Yu Tsuyusaki, Mio Tanaka, Yukichi Tanaka, Akihiro Hashiguchi, Hiroshi Takashima, Tomohide Goto
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is characterized by severe respiratory failure due to diaphragmatic paralysis and distal muscular weakness in early infancy. After an initial decline in respiratory state and motor function until 1-2years of age, residual capabilities reach a plateau. We report the peripheral neuropathological findings of a patient with SMARD1 at 1year and 1month of age, when his muscle strength and respiratory symptoms had deteriorated and then stabilized for several months...
September 9, 2017: Brain & Development
https://www.readbyqxmd.com/read/28890682/impaired-mitophagy-plays-a-role-in-denervation-of-neuromuscular-junctions-in-als-mice
#16
Robert S Rogers, Sudheer Tungtur, Tomohiro Tanaka, Lisa L Nadeau, Yomna Badawi, Hua Wang, Hong-Min Ni, Wen-Xing Ding, Hiroshi Nishimune
Motor neurons in amyotrophic lateral sclerosis (ALS) patients and animal models show degeneration from the nerve terminal, known as dying-back neuropathy. To investigate the mechanism underlying this neuropathy, we analyzed the neuromuscular junctions (NMJs) and motor neuron cell bodies in SOD1(G93A) mice using electron microscopy. NMJs of SOD1(G93A) mice exhibited significantly higher numbers of autophagosomes and degenerated mitochondria compared to wild-type controls. Mitophagosomes were identified in the NMJ presynaptic terminals of wild-type mice and SOD1(G93A) mice...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28888534/psychiatric-phenotypes-in-chronic-traumatic-encephalopathy
#17
REVIEW
Ian Mahar, Michael L Alosco, Ann C McKee
Chronic traumatic encephalopathy (CTE) is a neurodegenerative disorder involving cognitive, motor, and psychiatrically-relevant symptoms resulting from repetitive head impacts. Psychiatric phenotypes of CTE, including depression and suicidality, present particular challenges for CTE research, given that the diagnosis requires postmortem neuropathological examination. The pathognomonic lesion of CTE is the perivascular accumulation of hyperphosphorylated tau (ptau) protein at the depths of cortical sulci. These lesions are found in the earliest disease stages, and with advancing pathological severity, ptau deposition occurs in widespread brain regions in a four-stage scheme of severity...
September 6, 2017: Neuroscience and Biobehavioral Reviews
https://www.readbyqxmd.com/read/28883787/investigations-into-hypoxia-and-oxidative-stress-at-the-optic-nerve-head-in-a-rat-model-of-glaucoma
#18
Glyn Chidlow, John P M Wood, Robert J Casson
The vascular hypothesis of glaucoma proposes that retinal ganglion cell axons traversing the optic nerve head (ONH) undergo oxygen and nutrient insufficiency as a result of compromised local blood flow, ultimately leading to their degeneration. To date, evidence for the hypothesis is largely circumstantial. Herein, we made use of an induced rat model of glaucoma that features reproducible and widespread axonal transport disruption at the ONH following chronic elevation of intraocular pressure. If vascular insufficiency plays a role in the observed axonal transport failure, there should exist a physical signature at this time point...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28877995/a-neuroprotective-agent-that-inactivates-prodegenerative-trka-and-preserves-mitochondria
#19
Konstantin Feinberg, Adelaida Kolaj, Chen Wu, Natalie Grinshtein, Jonathan R Krieger, Michael F Moran, Lee L Rubin, Freda D Miller, David R Kaplan
Axon degeneration is an early event and pathological in neurodegenerative conditions and nerve injuries. To discover agents that suppress neuronal death and axonal degeneration, we performed drug screens on primary rodent neurons and identified the pan-kinase inhibitor foretinib, which potently rescued sympathetic, sensory, and motor wt and SOD1 mutant neurons from trophic factor withdrawal-induced degeneration. By using primary sympathetic neurons grown in mass cultures and Campenot chambers, we show that foretinib protected neurons by suppressing both known degenerative pathways and a new pathway involving unliganded TrkA and transcriptional regulation of the proapoptotic BH3 family members BimEL, Harakiri,and Puma, culminating in preservation of mitochondria in the degenerative setting...
September 6, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28867898/cortical-oligodendrocytes-in-kaolin-induced-hydrocephalus-in-wistar-rat-impact-of-degree-and-duration-of-ventriculomegaly
#20
Olugbenga Ayodeji Ayannuga, Thajasvarie Naicker
BACKGROUND: Oligodendrocytes are critical to the function of the brain. They generate the myelin sheath which ensures saltatory conduction, which is a more energy saving and efficient means of axonal impulse transmission. Ventriculomegaly results in neuronal degeneration and astrogliosis. PURPOSE: The effect of the degree of ventriculomegaly on oligodendrocyte in kaolin-induced hydrocephalus and the timeline have not been extensively documented, hence this study...
July 2017: Annals of Neurosciences
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