Results 31 to 40 of about 1,690 (150)

Stiripentol fails to lower plasma oxalate in a dialysis-dependent PH1 patient [PDF]

, 2020
Background: Primary hyperoxaluria type 1 (PH1) is a multisystemic metabolic disorder caused by an excessive production of oxalate by the liver. The majority of patients presenting in early infancy have end-stage renal disease (ESRD).
Müller-Schlüter, Karen, Knauf, Felix, Holle, Johannes, Kempf, Caroline, Pfau, Anja, Bufler, Philip, Müller, Dominik   +6 more
core   +1 more source

Therapeutic Drug Monitoring of the Newer Anti-Epilepsy Medications

Pharmaceuticals, 2010
In the past twenty years, 14 new antiepileptic drugs have been approved for use in the United States and/or Europe. These drugs are eslicarbazepine acetate, felbamate, gabapentin, lacosamide, lamotrigine, levetiracetam, oxcarbazepine, pregabalin ...
Matthew D. Krasowski
doaj   +1 more source

The Genetic Facets of Dravet Syndrome: Recent Insights [PDF]

Annals of Child Neurology
Dravet syndrome (DS), previously known as severe myoclonic epilepsy of infancy, is a severe epileptic syndrome affecting children, with an incidence of 1/22,000 to 1/49,900 live births annually. Characterized by resistant and prolonged seizures, it often
Hinde El Mouhi, Meriame Abbassi, Meryem Jalte, Abdelhafid Natiq, Laila Bouguenouch, Sana Chaouki   +5 more
doaj   +1 more source

Stiripentol protects against calcium oxalate nephrolithiasis and ethylene glycol poisoning

, 2019
International audienceIncreased urinary oxalate excretion (hyperoxaluria) promotes the formation of calcium oxalate crystals. Monogenic diseases due to hepatic enzyme deficiency result in chronic hyperoxaluria, promoting end-stage renal disease in ...
Joëlle Perez, Le Dudal, Marine, Jean-Philippe Haymann, Deschênes, Georges, Michel Daudon, Nicole Chemaly, Haymann, Jean-Philippe, Emmanuel Letavernier, Bouderlique, Elise, Carole Martori, Perez, Joëlle, Léa Huguet, Rima Nabbout, Elise Bouderlique, Tang, Ellie, Baud, Laurent, Daudon, Michel, Vandermeersch, Sophie, Frochot, Vincent, Laurent Baud, Martori, Carole, Georges Deschênes, Sophie Vandermeersch, Vincent Frochot, Chemaly, Nicole, Nabbout, Rima, Letavernier, Emmanuel, Marine Le Dudal, Huguet, Léa, Ellie Tang   +29 more
core   +1 more source

[Not Quite] The Ketogenic Diet in a Pill

Pediatric Neurology Briefs, 2015
Researchers at Okayama University, Japan showed lactate dehydrogenase (LDH) inhibition suppresses neuronal excitation in vitro, reduces EEG discharges and seizures in rodent models, and may provide a novel mechanism for anticonvulsant medications in ...
Andrew J Kim
doaj   +1 more source

Lipase Catalysed Kinetic Resolution of Stiripentol

, 2016
Kinetic resolution of rac-Stiripentol, catalysed by lipase A from Candida antarctica by esterification with vinyl butanoate was performed with an E-value of 24.
Aboul-Enein, Mohamed Nabil, Sundby, Eirik, Anthonsen, Thorleif, Abdel-Rehim, Mohamed, El-Behairy, Mohammed Farrag, Attia, Mohamed Ibrahim, El-Azzouny, Aida Abd El-Sattar, Amin, Kamilia M., Jacobsen, Elisabeth Egholm   +8 more
core   +1 more source

Stiripentol for focal refractory epilepsy

, 2015
BACKGROUND: This is an updated version of the original Cochrane review published in 2014 (Issue 1). For nearly 30% of people with epilepsy, seizures are not controlled by current treatments.
Igwe, Stanley C., BRIGO, Francesco, STORTI, Monica   +2 more
core   +1 more source

Fenfluramine for Treatment-Resistant Seizures in Patients With Dravet Syndrome Receiving Stiripentol-Inclusive Regimens A Randomized Clinical Trial [PDF]

, 2020
IMPORTANCE Fenfluramine treatment may reduce monthly convulsive seizure frequency in patients with Dravet syndrome who have poor seizure control with their current stiripentol-containing antiepileptic drug regimens.
Morrison, G. (Glenn), Mistry, A. (Arun), Knupp, K. (Kelly), Nabbout, R. (Rima), Farfel, G. (Gail), Villeneuve, N. (Nathalie), Stephani, U. (Ulrich), Laux, L. (Linda), Chiron, C. (Catherine), Auvin, S. (Stéphane), Perry, M.S. (M. Scott), Galer, B.S. (Bradley S.), Lock, M. (Michael), Wirrell, E. (Elaine), Zuberi, S. (Sameer), Agarwal, A. (Anupam), Sanchez-Carpintero, R. (Rocío)   +16 more
core   +3 more sources

Lactate dehydrogenases promote glioblastoma growth and invasion via a metabolic symbiosis

EMBO Molecular Medicine, 2022
Lactate is a central metabolite in brain physiology but also contributes to tumor development. Glioblastoma (GB) is the most common and malignant primary brain tumor in adults, recognized by angiogenic and invasive growth, in addition to its altered ...
Joris Guyon, Ignacio Fernandez‐Moncada, Claire M Larrieu, Cyrielle L Bouchez, Antonio C Pagano Zottola, Johanna Galvis, Tiffanie Chouleur, Audrey Burban, Kevin Joseph, Vidhya M Ravi, Heidi Espedal, Gro Vatne Røsland, Boutaina Daher, Aurélien Barre, Benjamin Dartigues, Slim Karkar, Justine Rudewicz, Irati Romero‐Garmendia, Barbara Klink, Konrad Grützmann, Marie‐Alix Derieppe, Thibaut Molinié, Nina Obad, Céline Léon, Giorgio Seano, Hrvoje Miletic, Dieter Henrik Heiland, Giovanni Marsicano, Macha Nikolski, Rolf Bjerkvig, Andreas Bikfalvi, Thomas Daubon   +31 more
doaj   +1 more source

Lactate‐Induced K370 Lactylation of STING Inhibits STING‐TBK1 Signaling and Dampens Anti‐Tumor Immunity

Advanced Science, EarlyView.
Lactate accumulation in glioblastoma drives lactylation of STING at lysine 370, a primate‐conserved residue. K370 lactylation weakens STING‐TBK1 binding, destabilizes STING, and disrupts ER‐to‐Golgi trafficking, blunting type I interferon signaling. LDHA inhibition reverses K370 lactylation and improves the antitumor efficacy of STING agonism combined ...
Yueyao Wu, Jingzhe Wang, Xu Chen, Yuntong Yang, Ping Wei, Han Xie, Honghao Wang, Chunyu Zhang, Siyi Xu, Qi Wang, Chunlong Zhong, Jing Zhang   +11 more
wiley   +1 more source