Results 41 to 50 of about 2,210 (155)
In order to increase the tissue level of tetrahydrobiopterin (BH4), supplementation with 6R-tetrahydrobiopterin (6RBH4) has been widely employed. In this work, the effectiveness of 6RBH4 was compared with 7,8-dihydrobiopterin (7,8BH2) and sepiapterin by ...
Keiko Sawabe +2 more
doaj +1 more source
Inherited metabolic epilepsies–established diseases, new approaches
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley +1 more source
Proteo‐transcriptomic insights into the venom composition of the ectoparasitoid Eupelmus urozonus
Integrated venom‐gland transcriptomics and venom proteomics of Eupelmus urozonus identified 316 venom proteins, including 75 secreted candidates, revealing diverse hydrolases, proteases/inhibitors and many uncharacterized effectors. Abstract BACKGROUND Ectoparasitoid venoms are ‘cocktails’ of bioactive molecules injected during oviposition to paralyze ...
Federica De Stefano +7 more
wiley +1 more source
ABSTRACT The aim of this study was to characterize the proteome of sperm and seminal plasma (SP) from rams with high semen freezability (HF) and low semen freezability (LF). Eleven ejaculates were collected from 12 rams, at 3‐day intervals, and cryopreserved (132 semen samples).
Wallisson Bruno de Morais Pacheco +14 more
wiley +1 more source
Sepiapterin Reductase Mediates Chemical Redox Cycling in Lung Epithelial Cells
In the lung, chemical redox cycling generates highly toxic reactive oxygen species that can cause alveolar inflammation and damage to the epithelium, as well as fibrosis.
Yang, Shaojun +6 more
core +1 more source
A new method for assay of sepiapterin deaminase (3.5.4.24) activity by use of high performance liquid chromatography (HPLC) was developed. By this sensitive method the enzyme activity in the cell organelle was assayed.
Tsusué M., Kuroda S., Sawada H.
doaj +1 more source
Vitamin‐Responsive Disorders: From Molecular Basis to Clinical Presentation and Therapy
ABSTRACT Vitamin‐dependent cofactors are essential for numerous metabolic reactions, and defects affecting their uptake, conversion, utilisation, or regeneration constitute a heterogeneous group of inherited metabolic disorders (IMDs). Although dietary vitamin intake is sufficient to sustain coenzyme synthesis in healthy individuals, it is insufficient
Cécile Acquaviva +5 more
wiley +1 more source
Sulfa Drugs Inhibit Sepiapterin Reduction and Chemical Redox Cycling by Sepiapterin Reductase [PDF]
Sepiapterin reductase (SPR) catalyzes the reduction of sepiapterin to dihydrobiopterin (BH2), the precursor for tetrahydrobiopterin (BH4), a cofactor critical for nitric oxide biosynthesis and alkylglycerol and aromatic amino acid metabolism.
Vladimir Mishin +8 more
core
Sepiapterin does not reduce albuminuria in diabetic apoE KO mice.
(A) PAS-stained glomeruli of apoE KO mice (apoE), diabetic apoE KO mice (DM) and diabetic apoE KO mice treated with sepiapterin (DM + S), showing heterogeneous diabetic lesions 14 weeks after induction of diabetes with STZ (20).
M. Cristina Avramut (3684979) +5 more
core +1 more source
Two Greek siblings with sepiapterin reductase deficiency
BACKGROUND: Sepiapterin reductase (SR) deficiency is a rare inherited disorder of neurotransmitter metabolism; less than 25 cases have been described in the literature so far.
Thöny, B; https://orcid.org/ +8 more
core +2 more sources

