Results 81 to 90 of about 150,374 (260)

The physiological and pathological effects of sphingolipid metabolism and signaling in the central nervous system

Brain Pathology, EarlyView.
Sphingolipids are vital components of cell membranes. Metabolic disruptions of sphingolipids, including ceramide and sphingosine‐1‐phosphate, are linked to neurological disorders. This article summarizes the classification, structure, and metabolic processes of sphingolipids, and the physiological and pathological effects of sphingolipid metabolism and
Tian Li, Haoying He, Ejuan Zhang, Fengjiao Hu, Zhuo Wang, Jian Xu, Mengliu Zeng, Biwen Peng   +7 more
wiley   +1 more source

Inhibition of O-acetylserine sulfhydrylase by fluoroalanine derivatives

Journal of Enzyme Inhibition and Medicinal Chemistry, 2018
O-acetylserine sulfhydrylase (OASS) is the pyridoxal 5′-phosphate dependent enzyme that catalyses the formation of L-cysteine in bacteria and plants. Its inactivation is pursued as a strategy for the identification of novel antibiotics that, targeting ...
Nina Franko, Konstantinos Grammatoglou, Barbara Campanini, Gabriele Costantino, Aigars Jirgensons, Andrea Mozzarelli   +5 more
doaj   +1 more source

Effect of 3‐mercaptopyruvate sulfurtransferase (3‐MST) inhibitors on contractile responses in porcine coronary artery

British Journal of Pharmacology, EarlyView.
Background and Purpose Hydrogen sulphide (H2S) is synthesised endogenously through cystathionine β‐synthase (CBS), cystathionine γ‐lyase (CSE) and 3‐mercaptopyruvate sulfurtransferase (3‐MST). Although exogenous H2S is known to produce vasodilatation, the vascular effect of H2S produced through 3‐MST is unknown. In this study, we demonstrate the effect
Maha Almaheize, Sahar Alharthi, Patricia Hemp, Jennifer Mattos, Marcus van Leer, Michael Garle, Stephen Alexander, Richard Roberts   +7 more
wiley   +1 more source

First‐in‐class inhibitors of SbnA reduce siderophore production in Staphylococcus aureus

The FEBS Journal, EarlyView.
The production of the iron‐chelating compound staphyloferrin B by the most invasive S. aureus strains is crucial for colonization, virulence, and survival in the host. Staphyloferrin B biosynthesis is carried out by eight enzymes, the Sbn system, that offer an appealing source of potential targets for the development of antimicrobials.
Sarah Hijazi, Monica Cozzi, Somayeh Asgharpour, Omar De Bei, Serena Faggiano, Francesco Marchesani, Luca Ronda, Marialaura Marchetti, Eleonora Gianquinto, Mariacristina Failla, Gauthier Trèves, Loretta Lazzarato, Francesca Spyrakis, Barbara Campanini, Emanuela Frangipani, Stefano Bettati   +15 more
wiley   +1 more source

The disease‐linked R336C mutation in cystathionine β‐synthase disrupts communication with the PLP cofactor, yet maintains the enzyme's overall structural integrity

The FEBS Journal, EarlyView.
Cystathionine β‐synthase (CBS) is an essential enzyme involved in cysteine metabolism. The mutation R336C, associated with severe homocystinuria and of high incidence in Qatar, reduces its activity. Our research shows that this mutation does not destabilize the enzyme fold but subtly alters the tautomeric equilibrium of the PLP cofactor within the ...
Carolina Conter, Reyes Núñez‐Franco, Duaa Walid Al‐Sadeq, Carmen Fernández‐Rodríguez, Naroa Goikoetxea‐Usandizaga, Gheyath K. Nasrallah, Michail Nomikos, Maria Luz Martinez‐Chantar, Alessandra Astegno, Gonzalo Jiménez‐Osés, Luis Alfonso Martínez‐Cruz   +10 more
wiley   +1 more source

Late-onset drug-resistant epilepsy in pyridoxamine 5′-phosphate oxidase deficiency: a case report

Journal of Medical Case Reports
Background Pyridoxamine 5′-phosphate oxidase deficiency is a rare inborn error of vitamin B6 metabolism that presents with drug-resistant epileptic seizures.
Erfan Naghavi, Afshan Davari, Amir Reza Bahadori, Mohammad Razmafrooz, Hamed AmiriFard, Iman Sabzgolin, Abbas Tafakhori, Mehrdad Sheikhvatan, Sara Ranji   +8 more
doaj   +1 more source

Reconstitution of the pyridoxal 5'-phosphate (PLP) dependent enzyme serine palmitoyltransferase (SPT) with pyridoxal reveals a crucial role for the phosphate during catalysis.

Chemical Communications, 2013
The pyridoxal 5'-phosphate (PLP)-dependent enzyme serine palmitoyltransferase (SPT) is required for de novo sphingolipid biosynthesis. A previous study revealed a novel and unexpected interaction between the hydroxyl group of the l-serine substrate and ...
A. E. Beattie, D. Clarke, J. M. Wadsworth, J. Lowther, Hochul Sin, D. Campopiano   +5 more
semanticscholar   +1 more source

Levodopa‐Responsive Dystonia Secondary to CTNNB1 Neurodevelopmental Disorder

Movement Disorders Clinical Practice, EarlyView.
Hanin Algethami, Wei Kang Lim, David Chitayat, Ingrid Tein, Alfonso Fasano, Carolina Gorodetsky   +5 more
wiley   +1 more source

The CRISPR‐Cas9 knockout DDC SH‐SY5Y in vitro model for AADC deficiency provides insight into the pathogenicity of R347Q and L353P variants: a cross‐sectional structural and functional analysis

The FEBS Journal, EarlyView.
Here, we generated a DDC‐KO cellular model for studying aromatic amino acid decarboxylase (AADC) deficiency. This model was created using CRISPR‐Cas9 in the SH‐SY5Y neuroblastoma cell line and was validated through the absence of the dopamine product and an altered l‐dopa metabolite profile.
Cristian Andres Carmona‐Carmona, Giovanni Bisello, Rossella Franchini, Gianluigi Lunardi, Roberta Galavotti, Massimiliano Perduca, Rui P. Ribeiro, Benny Danilo Belviso, Alejandro Giorgetti, Rocco Caliandro, Patricia M.‐J. Lievens, Mariarita Bertoldi   +11 more
wiley   +1 more source

Biomedical aspects of pyridoxal 5'-phosphate availability.

Frontiers in Bioscience, 2012
The biologically active form of vitamin B6, pyridoxal 5'-phosphate (PLP), is a cofactor in over 160 enzyme activities involved in a number of metabolic pathways, including neurotransmitter synthesis and degradation.
M. D. di Salvo, M. Safo, R. Contestabile
semanticscholar   +1 more source