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https://www.readbyqxmd.com/read/27915035/hyperinsulinaemic-hypoglycaemia-in-children-and-adults
#1
REVIEW
Pratik Shah, Sofia A Rahman, Huseyin Demirbilek, Maria Güemes, Khalid Hussain
Pancreatic β cells are functionally programmed to release insulin in response to changes in plasma glucose concentration. Insulin secretion is precisely regulated so that, under normal physiological conditions, fasting plasma glucose concentrations are kept within a narrow range of 3·5-5·5 mmol/L. In hyperinsulinaemic hypoglycaemia, insulin secretion becomes dysregulated (ie, uncoupled from glucose metabolism) so that insulin secretion persists in the presence of low plasma glucose concentrations. Hyperinsulinaemic hypoglycaemia is the most common cause of severe and persistent hypoglycaemia in neonates and children...
November 30, 2016: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/27902448/rasgrp1-promotes-amphetamine-induced-motor-behavior-through-a-rhes-interaction-network-rhesactome-in-the-striatum
#2
Neelam Shahani, Supriya Swarnkar, Vincenzo Giovinazzo, Jenny Morgenweck, Laura M Bohn, Catherina Scharager-Tapia, Bruce Pascal, Pablo Martinez-Acedo, Kshitij Khare, Srinivasa Subramaniam
The striatum of the brain coordinates motor function. Dopamine-related drugs may be therapeutic to patients with striatal neurodegeneration, such as Huntington's disease (HD) and Parkinson's disease (PD), but these drugs have unwanted side effects. In addition to stimulating the release of norepinephrine, amphetamines, which are used for narcolepsy and attention-deficit/hyperactivity disorder (ADHD), trigger dopamine release in the striatum. The guanosine triphosphatase Ras homolog enriched in the striatum (Rhes) inhibits dopaminergic signaling in the striatum, is implicated in HD and L-dopa-induced dyskinesia, and has a role in striatal motor control...
November 15, 2016: Science Signaling
https://www.readbyqxmd.com/read/27900521/-18-f-fluorodihydroxyphenylalanine-pet-ct-in-pheochromocytoma-and-paraganglioma-relation-to-genotype-and-amino-acid-transport-system-l
#3
Chloé C Feral, Floriane S Tissot, Lionel Tosello, Nicolas Fakhry, Fréderic Sebag, Karel Pacak, David Taïeb
PURPOSE: F-FDOPA is a highly sensitive and specific radiopharmaceutical for pheochromocytoma and paraganglioma (PPGL) imaging. However, (18)F-FDOPA might be falsely negative in these tumors, especially those related to mutations in succinate dehydrogenase genes (SDHx). The aim of the present study was to evaluate the relationship between expression of L-DOPA transporters and (18)F-FDOPA PET imaging results in PPGL. METHODS: From 2007 to 2015, 175 patients with non-metastatic PPGL were evaluated by (18)F-FDOPA PET/CT for initial diagnosis/staging and follow-up...
November 29, 2016: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/27898257/assessment-of-nifedipine-therapy-in-hyperinsulinemic-hypoglycemia-due-to-mutations-in-the-abcc8-gene
#4
M Güemes, P Shah, S Silvera, K Morgan, C Gilbert, L Hinchey, K Hussain
CONTEXT: Previous case reports have documented the effectiveness of L-type calcium channel blockers (such as Nifedipine and Verapamil) for treating different forms of hyperinsulinemic hypoglycemia (HH). OBJECTIVE: To systematically assess the glycemic response to Nifedipine therapy in 11 patients with HH due to mutations in the ABCC8 gene. DESIGN: Dose escalation of Nifedipine therapy Setting: Inpatients at a tertiary hospital Patients or Other Participants: 11 children with diazoxide unresponsive HH due to mutations in the ABCC8 gene...
November 29, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27897091/inducing-mutations-through-%C3%AE-irradiation-in-seeds-of-mucuna-pruriens-for-developing-high-l-dopa-yielding-genotypes
#5
Susheel Kumar Singh, Deepti Yadav, Raj Kishori Lal, Madan M Gupta, Sunita Singh Dhawan
PURPOSE: To develop elite genotypes in Mucuna pruriens (L.) DC with high L-DOPA (L-3, 4 dihydroxyphenylalanine) yields, with non-itching characteristics and better adaptability by applying γ-irradiation. Molecular and chemical analysis was performed for screening based on specific characteristics desired for developing suitable genotypes. MATERIALS AND METHODS: Developed, mutant populations were analyzed for L-DOPA % in seeds through TLC (thin layer chromatography), and the results obtained were validated with the HPLC (High performance liquid chromatography)...
November 29, 2016: International Journal of Radiation Biology
https://www.readbyqxmd.com/read/27889470/delayed-administration-of-dopaminergic-drugs-is-not-associated-with-prolonged-length-of-stay-of-hospitalized-patients-with-parkinson-s-disease
#6
Rob Skelly, Lisa Brown, Andrew Fogarty
BACKGROUND: Punctual delivery of dopaminergic medication to Parkinson's disease (PD) patients may be important in optimizing disease control. We tested the hypothesis that prompt delivery of l-dopa medications to emergency hospital inpatients was associated with a decreased length of stay in hospital. METHODS: The study population consisted of all urgent hospitalizations for patients with a diagnosis of PD to the Royal Derby Hospital over a two-year period. Data were extracted on timing of delivery of drugs, number of co-morbidities and length of stay...
November 14, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/27886621/evaluation-of-two-nucleophilic-syntheses-routes-for-the-automated-synthesis-of-6-18-f-fluoro-l-dopa
#7
Marc Pretze, Dominic Franck, Falk Kunkel, Erich Foßhag, Carmen Wängler, Björn Wängler
Two different strategies for the nucleophilic radiosynthesis of [(18)F]F-DOPA were evaluated regarding their applicability for an automated routine production on an Ecker&Ziegler Modular-Lab Standard module. Initially, we evaluated a promising 5-step synthesis based on a chiral, cinchonidine-derived phase-transfer catalyst (cPTC) being described to give the product in high radiochemical yields (RCY), high specific activities (AS) and high enantiomeric excesses (ee). However, the radiosynthesis of [(18)F]F-DOPA based on this strategy showed to be highly complex, giving the intermediate products as well as the final product in insufficient yields for automatization...
October 27, 2016: Nuclear Medicine and Biology
https://www.readbyqxmd.com/read/27882828/morinda-citrifolia-mitigates-rotenone-induced-striatal-neuronal-loss-in-male-sprague-dawley-rats-by-preventing-mitochondrial-pathway-of-intrinsic-apoptosis
#8
S Narasimhan Kishore Kumar, Jayakumar Deepthy, Uthamaraman Saraswathi, Mohan Thangarajeswari, Sathyamoorthy Yogesh Kanna, Pannerselvam Ezhil, Periandavan Kalaiselvi
OBJECTIVES: Parkinson disease (PD) is a neurodegenerative disorder affecting mainly the motor system, as a result of death of dopaminergic neurons in the substantia nigra pars compacta. The present scenario of research in PD is directed to identify novel molecules that can be administered individually or co-administered with L-Dopa to prevent the L-Dopa-Induced Dyskinesia (LID) like states that arise during chronic L-Dopa administration. Hence, in this study, we investigated whether Morinda citrifolia has therapeutic effects in rotenone-induced Parkinson's disease (PD) with special reference to mitochondrial dysfunction mediated intrinsic apoptosis...
November 24, 2016: Redox Report: Communications in Free Radical Research
https://www.readbyqxmd.com/read/27881231/burkholderia-pseudomallei-%C3%AE-carbonic-anhydrase-is-strongly-activated-by-amino-acids-and-amines
#9
Daniela Vullo, Sonia Del Prete, Sameh M Osman, Zeid AlOthman, Clemente Capasso, William A Donald, Claudiu T Supuran
Activation of the γ-class carbonic anhydrase (CAs, EC 4.2.1.1) from the pathogenic bacterium Burkholderia pseudomallei (BpsγCA) with a series of natural and non-natural amino acids and aromatic/heterocyclic amines has been investigated. The best BpsγCA activators were d-His, l-DOPA, d-Trp, 4-amino-l-Phe, dopamine, 2-(2-aminoethyl)pyridine, 2-aminoethyl-piparazine/morpholine and l-adrenaline, which showed activation constants ranging between 9 and 86nM. The least effective activators were l-His, l-Phe and 2-pyridyl-methylamine, with KAs in the range of 1...
November 12, 2016: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/27879548/switching-l-dopa-therapy-from-pulsatile-to-pulse-administration-reduces-motor-complications-in-parkinson-s-disease
#10
Giovanni Mostile, Alessandra Nicoletti, Valeria Dibilio, Antonina Luca, Loredana Raciti, Giorgia Sciacca, Calogero Edoardo Cicero, Rosario Vasta, Giulia Donzuso, Donatella Contrafatto, Mario Zappia
OBJECTIVE: To evaluate the severity of wearing-off and dyskinesia in patients with complicated Parkinson disease (PD) after switching L-dopa oral therapy from a "pulsatile" administration, consisting in intermittent multiple daily small doses of the drug, to a "pulse" administration, consisting in standard oral doses given at specific interdose intervals. METHODS: Thirty-four PD patients with motor complications were monitored twice with standardized waking day motor status evaluations using the Unified Parkinson Disease Rating Scale-Motor Examination (UPDRS-ME) and the Abnormal Involuntary Movement Scale (AIMS) after switching L-dopa administration modality from "pulsatile" to "pulse...
November 22, 2016: Clinical Neuropharmacology
https://www.readbyqxmd.com/read/27872930/the-inhibition-effect-of-starch-nanoparticles-on-tyrosinase-activity-and-its-mechanism
#11
Jie Yang, Ranran Chang, Shengju Ge, Mei Zhao, Caifeng Liang, Liu Xiong, Qingjie Sun
The objective of the current research was to investigate the effects of starch nanoparticles (SNPs) prepared from waxy maize, potato, normal corn, and tapioca starches on the activity of tyrosinase. As a main polyphenol oxidase, tyrosinase not only induces fruit and vegetable browning but also causes skin diseases by overproducing melanin. Herein, for the first time, we evaluated the inhibitory kinetics of SNPs on tyrosinase. It turned out that SNPs inhibited tyrosinase activity reversibly. The IC50 values of hollow nanoparticles, amylopectin nanoparticles, corn starch nanoparticles, and tapioca starch nanoparticles were 0...
November 22, 2016: Food & Function
https://www.readbyqxmd.com/read/27869329/novel-compound-heterozygous-synaptojanin-1-mutation-causes-l-dopa-responsive-dystonia-parkinsonism-syndrome
#12
Marc-Alexander Rauschendorf, Meike Jost, Friedrich Stock, Andreas Zimmer, Bernd Rösler, Michel Rijntjes, Tobias Piroth, Volker Arnd Coenen, Peter Christoph Reinacher, Philipp T Meyer, Lars Frings, Cornelius Weiller, Judith Fischer, Stephan Klebe
No abstract text is available yet for this article.
November 21, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27864045/influence-of-chronic-l-dopa-treatment-on-immune-response-following-allogeneic-and-xenogeneic-graft-in-a-rat-model-of-parkinson-s-disease
#13
Ludivine S Breger, Korbinian Kienle, Gaynor A Smith, Stephen B Dunnett, Emma L Lane
Although intrastriatal transplantation of fetal cells for the treatment of Parkinson's disease had shown encouraging results in initial open-label clinical trials, subsequent double-blind studies reported more debatable outcomes. These studies highlighted the need for greater preclinical analysis of the parameters that may influence the success of cell therapy. While much of this has focused on the cells and location of the transplants, few have attempted to replicate potentially critical patient centered factors...
November 15, 2016: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/27859864/l-dopa-induced-dyskinesia-and-neuroinflammation-do-microglia-and-astrocytes-play-a-role
#14
Anna R Carta, Giovanna Mulas, Mariza Bortolanza, Terence Duarte, Elisabetta Pillai, Gilberto Fisone, Rita Vozari Raisman, Elaine Del Bel
In Parkinson's disease (PD), L-DOPA therapy leads to the emergence of motor complications including L-DOPA-induced dyskinesia (LID). LID relies on a sequence of pre- and postsynaptic neuronal events, leading to abnormal corticostriatal neurotransmission and maladaptive changes in striatal projection neurons. In recent years, additional non-neuronal mechanisms have been proposed to contribute to LID. Among these mechanisms considerable attention has been focused on L-DOPA-induced inflammatory responses. Microglia and astrocytes are the main actors in neuroinflammatory responses, and their double role at the interface between immune and neurophysiological responses is starting to be elucidated...
November 17, 2016: European Journal of Neuroscience
https://www.readbyqxmd.com/read/27856221/levodopa-improves-learning-and-memory-ability-on-global-cerebral-ischemia-reperfusion-injured-rates-in-the-morris-water-maze-test
#15
Wenzhu Wang, Lixu Liu, Peng Jiang, Chen Chen, Tong Zhang
Previous studies have shown that levodopa (L-dopa) for 1-7days improved the consciousness level of certain patients who suffered from ischemia-reperfusion injury and were comatose for a long time period after cerebral resuscitation treatment. It also has an awakening effect on patients with disorders of consciousness. This study aimed to investigate whether L-dopa, which is used clinically to treat Parkinson's disease, might also ameliorate the behavior of rats following global cerebral ischemia-reperfusion injury...
November 14, 2016: Neuroscience Letters
https://www.readbyqxmd.com/read/27852786/electrophysiological-evidence-for-alternative-motor-networks-in-rem-sleep-behavior-disorder
#16
Marc Hackius, Esther Werth, Oguzkan Sürücü, Christian R Baumann, Lukas L Imbach
: Patients with Parkinson's disease (PD) and REM sleep behavior disorder (RBD) show mostly unimpaired motor behavior during REM sleep, which contrasts strongly to coexistent nocturnal bradykinesia. The reason for this sudden amelioration of motor control in REM sleep is unknown, however. We set out to determine whether movements during REM sleep are processed by different motor networks than movements in the waking state. We recorded local field potentials in the subthalamic nucleus (STN) and scalp EEG (modified 10/20 montage) during sleep in humans with PD and RBD...
November 16, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27843055/abnormal-premotor-motor-interaction-in-heterozygous-parkin-and-pink1-mutation-carriers
#17
Anne Weissbach, Tobias Bäumer, Peter P Pramstaller, Norbert Brüggemann, Vera Tadic, Robert Chen, Christine Klein, Alexander Münchau
OBJECTIVES: Mutations in the Parkin and PINK1 gene account for the majority of autosomal recessive early-onset Parkinson cases. There is increasing evidence that clinically asymptomatic subjects with single heterozygous mutations have a latent nigrostriatal dopaminergic deficit and could be taken as in vivo model of pre-symptomatic phase of Parkinsonism. METHODS: We charted premotor-motor excitability changes as compensatory mechanisms for subcortical dopamine depletions using transcranial magnetic stimulation by applying magnetic resonance-navigated premotor-motor cortex conditioning in 15 asymptomatic, heterozygous Parkin and PINK1 mutation carriers (2 female; mean age 53±8years) and 16 age- and sex-matched controls (5 female; mean age 57±9years)...
October 26, 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/27833521/treatment-responsive-holmes-tremor-case-report-and-literature-review
#18
REVIEW
Mohammed Alqwaifly
Holmes tremor is a rare symptomatic movement disorder, characterized by a combination of resting, postural, and action tremors. It is usually caused by lesions involving the brainstem, thalamus, and cerebellum. It is often difficult to treat, many medications have been used with varying degrees of success. It may respond to stereotactic thalamotomy and deep brain stimulation in ventralis intermedius nucleus. Here I report a case of Holmes tremor secondary to multiple sclerosis that treated with L-dopa/carpidopa and showed marked improvement...
October 2016: International Journal of Health Sciences
https://www.readbyqxmd.com/read/27829416/tyrosinase-inhibition-and-antioxidant-properties-of-asphodelus-microcarpus-extracts
#19
Amalia Di Petrillo, Ana Maria González-Paramás, Benedetta Era, Rosaria Medda, Francesca Pintus, Celestino Santos-Buelga, Antonella Fais
BACKGROUND: Asphodelus microcarpus belongs to the family Liliaceae that include several medicinal plants. In the traditional medicine plants of the genus Asphodelus are used to treat skin disorders such as ectodermal parasites, psoriasis, microbial infection and for lightening freckles. In order to find novel skin depigmenting agents, the present work was carry out to evaluate antioxidant activity and tyrosinase inhibitory potential of leaves, flowers and tubers extracts of A. microcarpus...
November 9, 2016: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/27826808/molecular-cloning-and-characteristic-features-of-a-novel-extracellular-tyrosinase-from-aspergillus-niger-pa2
#20
Pragati Agarwal, Jyoti Singh, R P Singh
Aspergillus niger PA2, a novel strain isolated from waste effluents of food industry, is a potential extracellular tyrosinase producer. Enzyme activity and L-DOPA production were maximum when glucose and peptone were employed as C source and nitrogen source respectively in the medium and enhanced notably when the copper was supplemented, thus depicting the significance of copper in tyrosinase activity. Tyrosinase-encoding gene from the fungus was cloned, and amplification of the tyrosinase gene yielded a 1127-bp DNA fragment and 374 amino acid residue long product that encoded for a predicted protein of 42...
November 8, 2016: Applied Biochemistry and Biotechnology
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