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1. Cerliponase alfa (Brineura) - the treatment of neuronal ceroid lipofuscinosis type 2 (CLN2) disease, also known as tripeptidyl peptidase 1 (TPP1) deficiency

Cerliponase alfa (Brineura) - the treatment of neuronal ceroid lipofuscinosis type 2 (CLN2) disease, also known as tripeptidyl peptidase 1 (TPP1) deficiency Search Page - Drug and Health Product Register Language selection Search and menus Search Search website Search Topics menu You are here: Summary Basis of Decision - - Health Canada Expand all Summary Basis of Decision (SBD) for Contact: Summary Basis of Decision (SBD) documents provide information related to the original authorization

2019 Health Canada - Drug and Health Product Register

2. Mapping neuronal inputs to Kiss1 neurons in the arcuate nucleus of the mouse. (PubMed)

Mapping neuronal inputs to Kiss1 neurons in the arcuate nucleus of the mouse. The normal function of the mammalian reproductive axis is strongly influenced by physiological, metabolic and environmental factors. Kisspeptin neuropeptides, encoded by the Kiss1 gene, are potent regulators of the mammalian reproductive axis by stimulating gonadodropin releasing hormone secretion from the hypothalamus. To understand how the reproductive axis is modulated by higher order neuronal inputs we have mapped (...) the afferent circuits into arcuate (ARC) Kiss1 neurons. We used a transgenic mouse that expresses the CRE recombinase in Kiss1 neurons for conditional viral tracing with genetically modified viruses. CRE-mediated activation of these viruses in Kiss1 neurons allows the virus to move transynaptically to label neurons with primary or secondary afferent inputs into the Kiss1 neurons. Several regions of the brain showed synaptic connectivity to arcuate Kiss1 neurons including proopiomelanocortin neurons

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2019 PLoS ONE

3. Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease. Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is terminal, progressive neurological condition for which there are no curative treatments. Among people with ALS/MND, fatigue is a common and debilitating symptom, which is characterised by reversible motor weakness and whole-body tiredness that is only partially relieved by rest. The effectiveness of pharmacological or non-pharmacological

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2018 Cochrane

5. Motor neurone disease: assessment and management

Motor neurone disease: assessment and management Motor neurone disease: assessment and Motor neurone disease: assessment and management management NICE guideline Published: 24 February 2016 nice.org.uk/guidance/ng42 © NICE 2019. All rights reserved. Subject to Notice of rights (https://www.nice.org.uk/terms-and-conditions#notice-of- rights).Y Y our responsibility our responsibility The recommendations in this guideline represent the view of NICE, arrived at after careful consideration (...) be inconsistent with complying with those duties. Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible. Motor neurone disease: assessment and management (NG42) © NICE 2019. All rights reserved. Subject to Notice of rights (https://www.nice.org.uk/terms-and- conditions#notice-of-rights). Page 2 of 48Contents Contents Overview 4 Who

2016 National Institute for Health and Clinical Excellence - Clinical Guidelines

6. Brineura - cerliponase alfa - Neuronal Ceroid Lipofuscinosis Type 2

Brineura - cerliponase alfa - Neuronal Ceroid Lipofuscinosis Type 2 cerliponase alfa | CADTH.ca Find the information you need cerliponase alfa cerliponase alfa Last Updated: June 28, 2019 Result type: Reports Project Number: SR0574-000 Product Line: Generic Name: cerliponase alfa Brand Name: Brineura Manufacturer: Biomarin Pharmaceutical (Canada) Inc. Indications: Neuronal Ceroid Lipofuscinosis Type 2 Manufacturer Requested Reimbursement Criteria 1 : Indicated for patients with CLN2 disease (...) , also known as tripeptidyl peptidase 1 (TPP1) deficiency or Neuronal Ceroid Lipofuscinosis type 2. Submission Type: New Project Status: Complete Biosimilar: No Companion Diagnostics: No Date Recommendation Issued: May 23, 2019 Recommendation Type: Reimburse with clinical criteria and/or conditions Fee Schedule: Schedule A The requested reimbursement criteria are provided by the applicant and do not necessarily reflect the views of CADTH. Reimbursement criteria from CADTH will be documented

2018 Canadian Agency for Drugs and Technologies in Health - Common Drug Review

7. Engrafted newborn neurons could functionally integrate into the host neuronal network (PubMed)

Engrafted newborn neurons could functionally integrate into the host neuronal network 28271666 2018 02 26 2018 11 13 2095-8137 38 1 2017 Jan 18 Zoological research Zool Res Engrafted newborn neurons could functionally integrate into the host neuronal network. 5-6 10.13918/j.issn.2095-8137.2017.005 Wang Zheng-Bo ZB Key Laboratory of Animal Models and Human Disease Mechanisms of Chinese Academy of Sciences & Yunnan Province, Kunming Institute of Zoology, Kunming Yunnan 650223, China. Qin Dong (...) Apr 24;157(3):726-39 24746791 Neuron. 2011 May 26;70(4):614-25 21609820 Cell Transplant. 2015;24(4):681-90 25839189 J Neurosci. 2004 Apr 28;24(17):4145-56 15115809 Nat Med. 1999 Dec;5(12):1410-2 10581084 Oxid Med Cell Longev. 2015;2015:618631 26146528 J Cereb Blood Flow Metab. 2016 Dec;36(12 ):2034-2043 27742890 Nature. 2002 Jul 4;418(6893):50-6 12077607 Nat Neurosci. 2013 Aug;16(8):1154-61 23792946 Int J Mol Sci. 2015 Nov 05;16(11):26473-92 26556344 Proc Natl Acad Sci U S A. 2011 Dec 13;108(50

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2017 Zoological research

8. A systematic review and meta-analysis: post-mortem hippocampal subfield volume, neuronal number, and neuronal density in schizophrenia and control subjects

A systematic review and meta-analysis: post-mortem hippocampal subfield volume, neuronal number, and neuronal density in schizophrenia and control subjects Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content

2019 PROSPERO

9. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common cause of death, which usually occurs within two to five years of the disease onset. Supporting respiratory function with mechanical ventilation may improve survival and quality of life. This is the second update of a review first published in 2009

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2017 Cochrane

10. Gamma aminobutyric acid (GABA) modulators for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Gamma aminobutyric acid (GABA) modulators for amyotrophic lateral sclerosis/motor neuron disease. Imbalance of gamma aminobutyric acid (GABA) and related modulators has been implicated as an important factor in the pathogenesis of amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND). In this context, the role and mechanism of action of gabapentin and baclofen have been extensively investigated, although with conflicting results. This is the first systematic

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2017 Cochrane

11. Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. Motor neuron disease (MND), which is also known as amyotrophic lateral sclerosis (ALS), causes a wide range of symptoms but the evidence base for the effectiveness of the symptomatic treatment therapies is limited.To summarise the evidence from Cochrane Systematic Reviews of all symptomatic treatments for MND.We searched the Cochrane Database of Systematic Reviews (CDSR) on 15 November 2016 for systematic reviews

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2017 Cochrane

12. Uncovering Neuronal Networks Defined by Consistent Between-Neuron Spike Timing from Neuronal Spike Recordings (PubMed)

Uncovering Neuronal Networks Defined by Consistent Between-Neuron Spike Timing from Neuronal Spike Recordings It is widely assumed that distributed neuronal networks are fundamental to the functioning of the brain. Consistent spike timing between neurons is thought to be one of the key principles for the formation of these networks. This can involve synchronous spiking or spiking with time delays, forming spike sequences when the order of spiking is consistent. Finding networks defined (...) by their sequence of time-shifted spikes, denoted here as spike timing networks, is a tremendous challenge. As neurons can participate in multiple spike sequences at multiple between-spike time delays, the possible complexity of networks is prohibitively large. We present a novel approach that is capable of (1) extracting spike timing networks regardless of their sequence complexity, and (2) that describes their spiking sequences with high temporal precision. We achieve this by decomposing frequency-transformed

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2018 eNeuro

13. [Cerliponase alfa (neuronal ceroid lipofuscinosis) - assessment according to õ35a (para. 1., sentence 10) Social Code Book V]

[Cerliponase alfa (neuronal ceroid lipofuscinosis) - assessment according to õ35a (para. 1., sentence 10) Social Code Book V] Cerliponase alfa (neuronale zeroidlipofuszinose typ 2): bewertung gemäß § 35a Abs. 1 Satz 10 SGB V; dossierbewertung; auftrag G17-06 [Cerliponase alfa (neuronal ceroid lipofuscinosis) - assessment according to §35a (para. 1., sentence 10) Social Code Book V] Cerliponase alfa (neuronale zeroidlipofuszinose typ 2): bewertung gemäß § 35a Abs. 1 Satz 10 SGB V (...) ; dossierbewertung; auftrag G17-06 [Cerliponase alfa (neuronal ceroid lipofuscinosis) - assessment according to §35a (para. 1., sentence 10) Social Code Book V] Institut für Qualität und Wirtschaftlichkeit im Gesundheitswesen Record Status This is a bibliographic record of a published health technology assessment from a member of INAHTA. No evaluation of the quality of this assessment has been made for the HTA database. Citation Institut für Qualität und Wirtschaftlichkeit im Gesundheitswesen. Cerliponase alfa

2018 Health Technology Assessment (HTA) Database.

14. Impact of insulin on primary arcuate neurons culture is dependent on early-postnatal nutritional status and neuronal subpopulation. (PubMed)

Impact of insulin on primary arcuate neurons culture is dependent on early-postnatal nutritional status and neuronal subpopulation. Nutrition plays a critical role in programming and shaping linear growth during early postnatal life through direct action on the development of the neuroendocrine somatotropic (GH/IGF-1) axis. IGF-1 is a key factor in modulating the programming of linear growth during this period. Notably, IGF-1 preferentially stimulates axonal growth of GHRH neurons (...) specific approach (GHRH-eGFP mice) under both physiological conditions (normally fed pups) and those of dietary restriction (underfed pups). Our data suggest that insulin failed to directly control axonal growth of Arc neurons or influence specific IGF-1-mediated effects on GHRH neurons. Insulin may act on neuronal welfare, which appears to be dependent on neuronal sub-populations and is influenced by the nutritional status of pups in which Arc neurons develop.

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2018 PLoS ONE

15. Expression-based decision tree model reveals distinct microRNA expression pattern in pediatric neuronal and mixed neuronal-glial tumors. (PubMed)

Expression-based decision tree model reveals distinct microRNA expression pattern in pediatric neuronal and mixed neuronal-glial tumors. The understanding of the molecular biology of pediatric neuronal and mixed neuronal-glial brain tumors is still insufficient due to low frequency and heterogeneity of those lesions which comprise several subtypes presenting neuronal and/or neuronal-glial differentiation. Important is that the most frequent ganglioglioma (GG) and dysembryoplastic

2019 BMC Cancer

16. USE of anti-neuronal antibodies to diagnose paraneoplastic neurological syndromes

USE of anti-neuronal antibodies to diagnose paraneoplastic neurological syndromes Use of anti-neuronal antibodies to diagnose paraneoplastic neurological syndromes Use of anti-neuronal antibodies to diagnose paraneoplastic neurological syndromes Gonzalez L, Augustovski F, Pichon-Riviere A, García Martí S, Alcaraz A, Bardach A, Ciapponi A, López A, Rey-Ares L Record Status This is a bibliographic record of a published health technology assessment from a member of INAHTA. No evaluation (...) of the quality of this assessment has been made for the HTA database. Citation Gonzalez L, Augustovski F, Pichon-Riviere A, García Martí S, Alcaraz A, Bardach A, Ciapponi A, López A, Rey-Ares L. Use of anti-neuronal antibodies to diagnose paraneoplastic neurological syndromes. Buenos Aires: Institute for Clinical Effectiveness and Health Policy (IECS). Informe de Respuesta Rapida No. 473. 2016 Authors' conclusions The evidence found supporting the use of anti-neuronal antibodies to diagnose paraneoplastic

2017 Health Technology Assessment (HTA) Database.

17. Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease. Amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND) is a fatal disease associated with rapidly progressive disability, for which no definitive treatment as yet exists. Current treatment regimens largely focus on relieving symptoms to improve the quality of life of those affected. Based on data from preclinical studies, cell-based therapy is a promising treatment for ALS/MND.To assess

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2016 Cochrane

18. BIG1 is required for the survival of deep layer neurons, neuronal polarity, and the formation of axonal tracts between the thalamus and neocortex in developing brain. (PubMed)

BIG1 is required for the survival of deep layer neurons, neuronal polarity, and the formation of axonal tracts between the thalamus and neocortex in developing brain. BIG1, an activator protein of the small GTPase, Arf, and encoded by the Arfgef1 gene, is one of candidate genes for epileptic encephalopathy. To know the involvement of BIG1 in epileptic encephalopathy, we analyzed BIG1-deficient mice and found that BIG1 regulates neurite outgrowth and brain development in vitro and in vivo (...) . The loss of BIG1 decreased the size of the neocortex and hippocampus. In BIG1-deficient mice, the neuronal progenitor cells (NPCs) and the interneurons were unaffected. However, Tbr1+ and Ctip2+ deep layer (DL) neurons showed spatial-temporal dependent apoptosis. This apoptosis gradually progressed from the piriform cortex (PIR), peaked in the neocortex, and then progressed into the hippocampus from embryonic day 13.5 (E13.5) to E17.5. The upper layer (UL) and DL order in the neocortex was maintained

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2017 PLoS ONE

19. Communications Technology and Motor Neuron Disease: An Australian Survey of People With Motor Neuron Disease (PubMed)

Communications Technology and Motor Neuron Disease: An Australian Survey of People With Motor Neuron Disease People with Motor Neuron Disease (MND), of which amyotrophic lateral sclerosis (ALS) is the most common form in adults, typically experience difficulties with communication and disabilities associated with movement. Assistive technology is essential to facilitate everyday activities, promote social support and enhance quality of life.This study aimed to explore the types of mainstream (...) and commonly available communication technology used by people with MND including software and hardware, to identify the levels of confidence and skill that people with MND reported in using technology, to determine perceived barriers to the use of technology for communication, and to investigate the willingness of people with MND to adopt alternative modes of communication.An on-line survey was distributed to members of the New South Wales Motor Neuron Disease Association (MND NSW). Descriptive techniques

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2016 JMIR rehabilitation and assistive technologies

20. hsa-let-7c miRNA Regulates Synaptic and Neuronal Function in Human Neurons (PubMed)

hsa-let-7c miRNA Regulates Synaptic and Neuronal Function in Human Neurons Non-coding RNA, including microRNA (miRNA) serves critical regulatory functions in the developing brain. The let-7 family of miRNAs has been shown to regulate neuronal differentiation, neural subtype specification, and synapse formation in animal models. However, the regulatory role of human let-7c (hsa-let-7c) in human neuronal development has yet to be examined. Let-7c is encoded on chromosome 21 in humans (...) and therefore may be overexpressed in human brains in Trisomy 21 (T21), a complex neurodevelopmental disorder. Here, we employ recent developments in stem cell biology to show that hsa-let-7c mediates important regulatory epigenetic functions that control the development and functional activity of human induced neuronal cells (iNs). We show that overexpression of hsa-let-7c in human iNs derived from induced pluripotent stem (iPS), as well as embryonic stem (ES), cells leads to morphological as well

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2018 Frontiers in synaptic neuroscience

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