Results 161 to 170 of about 13,139 (196)

Tetrahydrobiopterin-(BH4-)Mangelkrankheiten

2014
Die Krankheitsgruppe umfasst 6 angeborene Stoffwechselstorungen, die mit einem Tetrahydrobiopterinmangel (BH4-Mangel) entweder aufgrund einer gestorten BH4-Biosynthese oder eines gestorten BH4-Recyclings einhergehen. Tetrahydrobiopterin (BH4) ist ein essenzieller Kofaktor bei der Biosynthese der Neurotransmitter Dopamin und Serotonin und bei der ...
T. Opladen, N. Blau
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Molecular genetics of tetrahydrobiopterin (BH4) deficiency in the Maltese population

Molecular Genetics and Metabolism, 2007
Deficient activity of the Dihydropteridine Reductase enzyme (DHPR; EC 1.5.1.34; OMIM 261630) is due to mutations in the Quinoid Dihydropteridine Reductase gene on 4p15.3 (QDPR; RefSeq NM_000320). It results in defective recycling of tetrahydrobiopterin (BH(4)) and homozygotes have a rare form of atypical Hyperphenylalaninaemia and Phenylketonuria (aPKU)
Rosienne, Farrugia, Christian A, Scerri, Simon Attard, Montalto, Raymond, Parascandolo, Brian G R, Neville, Alex E, Felice   +5 more
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Disruption of tetrahydrobiopterin (BH4) biosynthesis pathway affects cuticle pigmentation in Henosepilachna vigintioctopunctata

Journal of Insect Physiology, 2023
Tetrahydrobiopterin (BH4) is produced from guanosine triphosphate (GTP) under catalyzation of GTP cyclohydrolase I (GTPCH), 6-pyruvoyltetrahydropterin synthase (PTPS) and sepiapterin reductase (SR), among others. In Drosophila melanogaster, BH4 and other pteridines are required for cuticle tanning and eye pigmentation.
Long-Ji, Ze, Ping, Xu, Jian-Jian, Wu, Lin, Jin, Ahmad, Ali Anjum, Guo-Qing, Li   +5 more
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The Effect of Tetrahydrobiopterin (BH4) on Sperm Motility

2002
Seminal plasma has the highest concentration of tetrahydrobiopterin (BH4) in human body fluids. This study evaluated the effect of BH4 in the seminal plasma on sperm motility.
H. Shintaku, Y. Sawada, Y. Nakamura, A. Muso, T. Yamano   +4 more
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Tetrahydrobiopterin (BH4) in septic shock

Critical Care Medicine, 2012
In the last few years, a growing body of evidence has linked the development of multiorgan failure and death in septic patients to the presence of microcirculatory abnormalities (1–3). These microvascular alterations still take place in spite of the normalization of systemic hemodynamics and ...
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Albino (al) is a tetrahydrobiopterin (BH4)-deficient mutant of the silkworm Bombyx mori

Insect Biochemistry and Molecular Biology, 2013
Albino (al) is a lethal mutant of Bombyx mori that exhibits a colourless cuticle after the first ecdysis and dies without feeding on mulberry. Previous studies have indicated that sclerotisation was insufficient because of defective phenylalanine and tyrosine metabolism in albino larvae.
Tsuguru, Fujii, Hiroaki, Abe, Munetaka, Kawamoto, Susumu, Katsuma, Yutaka, Banno, Toru, Shimada   +5 more
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Administration of Tetrahydrobiopterin (BH4) Protects the Renal Microcirculation From Ischemia and Reperfusion Injury

Anesthesia & Analgesia, 2017
BACKGROUND: Abdominal aortic aneurysm surgery with suprarenal cross-clamping is often associated with renal injury. Although the mechanism underlying such injury is unclear, tissue ischemia and reperfusion, which induces endothelial dysfunction and decreases the availability of tetrahydrobiopterin (BH4), may play a role.
Rahmania, Lokmane, Orbegozo Cortes, Diego, Su, FUHONG, Taccone, Fabio, Vincent, Jean Louis, De Backer, Daniel   +5 more
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The % Tetrahydrobiopterin (BH4) and Serine 1177 Phosphorylation Is Reduced in Sickle Transgenic Mice.

Blood, 2007
Abstract Tetrahydrobiopterin (BH4) is an essential co-factor of nitric oxide synthase (NOS), the enzyme that generates nitric oxide (NO) from arginine and oxygen. NO plays an essential role in maintaining vascular tone and endothelial function, reducing levels of adhesion molecules and platelet aggregation, and reducing inflammation; all
Qiuying Chen, Ashley Hale, Nicholas J. Alp, Keith M. Channon, Mary E. Fabry   +4 more
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[Tetrahydrobiopterin (BH4) deficiency - diagnosis and treatment].

Orvosi hetilap, 2018
Since the initial breaking discovery of Følling that the severe neurological consequences of phenylketonuria could be prevented by use of low phenylalanine (Phe) diet, it has been shortly recognised that defective phenylalanine metabolism may also arise from the deficiency of tetrahydrobiopterin (BH4) cofactor, required for phenylalanine-hydroxylase ...
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Administration of tetrahydrobiopterin (BH4) improves outcome in septic shock

2011
info:eu-repo/semantics ...
He, Xinrong, Su, FUHONG, Velissaris, Dimitrios, Salgado, Diamantino, de Souza Barros, Dalton, Lorent, Sophie, Taccone, Fabio Silvio, Vincent, Jean Louis, De Backer, Daniel   +8 more
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