Results 11 to 20 of about 1,140 (181)

Age-Dependent Variation in Blood Biopterin Peaks Following Oral Tetrahydrobiopterin Administration in Phenylketonuria. [PDF]

J Inherit Metab Dis
ABSTRACT The correct diagnosis of tetrahydrobiopterin (BH4, sapropterin dihydrochloride)‐responsive phenylketonuria (PKU) and treatment with BH4 are important for prognosis and quality of life. We examined whether age affects biopterin bioavailability following oral BH4 administration in PKU and whether this influences BH4 responsiveness.
Kitayama K, Sakaguchi T, Nakano N, Kabata D, Shintaku H, Hamazaki T.   +5 more
europepmc   +2 more sources

Guide RNA structure design enables combinatorial CRISPRa programs for biosynthetic profiling. [PDF]

Nat Commun
Engineering metabolism to efficiently produce chemicals from multi-step pathways requires optimizing multi-gene expression programs to achieve enzyme balance.
Fontana J, Sparkman-Yager D, Faulkner I, Cardiff R, Kiattisewee C, Walls A, Primo TG, Kinnunen PC, Garcia Martin H, Zalatan JG, Carothers JM.   +10 more
europepmc   +3 more sources

Mapping an atlas of tissue-specific drosophila melanogaster metabolomes by high resolution mass spectrometry [PDF]

, 2013
Metabolomics can provide exciting insights into organismal function, but most work on simple models has focussed on the whole organism metabolome, so missing the contributions of individual tissues.
A Kamleh, A Malmendal, A Malmendal, AA Jackson, C Jeulin, David G. Watson, DG Watson, DJ Creek, Dominika Korzekwa, DT Sullivan, G Di Paolo, G Fraenkel, GJ Blomquist, GX Quan, H Colinet, J Andrews, J Overgaard, Jacob Guy Bundy, JD Feala, JE Treherne, JL Hubby, JS Park, Julian A. T. Dow, KS Pedersen, L Coquin, L Pan, L Zheng, M Carvalho, M Parisi, M Rera, MA Kamleh, MA Kamleh, MF Wolfner, MM Green, Mohammed Al Bratty, MS Elias, N Vereshchagina, NE Braverman, P Niewiadomska, PA Robitaille, PS Shekhawat, R Tearle, S Buch, S Reisse, V Kostal, Venkateswara R. Chintapalli, VR Chintapalli, VR Chintapalli   +47 more
core   +3 more sources

Studies on the regulation of endothelial nitric oxide synthase in endothelial dysfunction [PDF]

, 2016
Ischaemic heart disease and cerebrovascular disease are the leading causes of morbidity and mortality in the world. The underlying progression of the disease is linked to a reduction in the bioavailability of nitric oxide. One factor contributing to this
Cerrato, Ruha
core   +1 more source

Insulin Reverses D-Glucose–Increased Nitric Oxide and Reactive Oxygen Species Generation in Human Umbilical Vein Endothelial Cells [PDF]

, 2015
Vascular tone is controlled by the L-arginine/nitric oxide (NO) pathway, and NO bioavailability is strongly affected by hyperglycaemia-induced oxidative stress.
Aguayo, Claudio, Avila, Pía, Cabrera, Lissette, González, Marcelo, Palma, Carlos, Rojas, Susana, Sobrevia Luarte, Luis, Villalobos, Roberto   +7 more
core   +1 more source

Plasmodium Infection Is Associated with Impaired Hepatic Dimethylarginine Dimethylaminohydrolase Activity and Disruption of Nitric Oxide Synthase Inhibitor/Substrate Homeostasis. [PDF]

, 2017
Inhibition of nitric oxide (NO) signaling may contribute to pathological activation of the vascular endothelium during severe malaria infection. Dimethylarginine dimethylaminohydrolase (DDAH) regulates endothelial NO synthesis by maintaining homeostasis ...
Ackerman, Hans, Alkaitis, Matthew S, Bilker, Oliver, Casals-Pascual, Climent, Chertow, Jessica H, Conway, David J., Correa, Simon, Cunnington, Aubrey J, Ebonyi, Augustine O, Ikeda, Allison K, Jayasooriya, Shamanthi, Nardone, Glenn, Njie, Madi, Okebe, Joseph, Walther, Michael   +14 more
core   +4 more sources

An Efficient Synthesis of 2'-O-(β-D-Ribofuranosyl)biopterin [PDF]

, 2008
N(2)-(N,N-Dimethylaminomethylene)-3-[2-(4-nitrophenyl)ethyl]-1',2'-di-O (trimethylsilyl)biopterin (4) was prepared from biopterin (1a, 86% overall yield) in 5 steps. Glycosylation of 4 with 1,2,3,5-tetra-O-acetyl-β D-ribofuranose (5a) and its 2,3,5-tri-O-
Fujita, Hiroshi, Hanaya, Tadashi, Pfleiderer, Wolfgang, Soranaka, Kazuyuki, Torigoe, Kiyoshi, Yamamoto, Hiroshi   +5 more
core   +1 more source

First Synthesis of Asperopterin A, an Isoxanthopterin Glycoside from Aspergillus Oryzae [PDF]

, 2013
The key precursor, N-2-(N,N -dimethylaminomethylene)-6-hydroxymethyl-8-methyl-3-[2-(4-nitrophenyl)ethyl]-7-xanthopterin (16) was efficiently prepared from 2,5-diamino-6-methylamino-3H-pyrimidin-4-one (5) and ethyl 3-(tert-butyldimethylsilyloxy)-2 ...
Hanaya, Tadashi, Yamamoto, Hiroshi
core   +1 more source

Biopterin responsive phenylalanine hydroxylase deficiency [PDF]

Genetics in Medicine, 2004
Phenylketonuria (PKU) is an autosomal recessive disorder caused by mutations in the phenylalanine hydroxylase (PAH) gene. There have been more than 400 mutations identified in the PAH gene leading to variable degrees of deficiency in PAH activity, and consequently a wide spectrum of clinical severity.
Reuben, Matalon, Richard, Koch, Kimberlee, Michals-Matalon, Kathryn, Moseley, Sankar, Surendran, Stephen, Tyring, Heidi, Erlandsen, Alejandra, Gamez, Raymond C, Stevens, Anne, Romstad, Lisbeth B, Møller, Flemming, Guttler   +11 more
openaire   +2 more sources

Inflammation and oxidative stress caused by nitric oxide synthase uncoupling might lead to left ventricular diastolic and systolic dysfunction in patients with hypertension [PDF]

, 2018
K
Bodó, Imre, Fazakas, Ádám, Fekete, Bertalan C., Kiss, Melinda, Molvarec, Attila, Nagy, Bálint, Nagy, Eszter, Patócs, Attila, Pepó, Lilla, Prohászka, Zoltán, Szelényi, Zsuzsanna, Szénási, Gábor, Tegze, Narcis, Vereckei, András   +13 more
core   +1 more source