[HTML][HTML] Crucial neuroprotective roles of the metabolite BH4 in dopaminergic neurons
Dopa-responsive dystonia (DRD) and Parkinson's disease (PD) are movement disorders
caused by the dysfunction of nigrostriatal dopaminergic neurons. Identifying druggable …
caused by the dysfunction of nigrostriatal dopaminergic neurons. Identifying druggable …
Mechanisms underlying the efficacy and limitation of dopa and tetrahydrobiopterin therapies for the deficiency of GTP cyclohydrolase 1 revealed in a novel mouse …
X Jiang, Y Shao, Y Liao, X Zheng, M Peng, Y Cai… - European Journal of …, 2024 - Elsevier
Dopa and tetrahydrobiopterin (BH4) supplementation are recommended therapies for the
dopa-responsive dystonia caused by GTP cyclohydrolase 1 (GCH1, also known as GTPCH) …
dopa-responsive dystonia caused by GTP cyclohydrolase 1 (GCH1, also known as GTPCH) …
GTP cyclohydrolase regulation: implications for brain development and function
H Ichinose, D Homma, C Sumi-Ichinose… - Advances in …, 2013 - Elsevier
Tetrahydrobiopterin (BH4) is essential for the biosynthesis of dopamine, noradrenaline, and
serotonin, which serve as cofactors for tyrosine hydroxylase (TH) and tryptophan …
serotonin, which serve as cofactors for tyrosine hydroxylase (TH) and tryptophan …
The neurobiology of tetrahydrobiopterin biosynthesis: a model for regulation of GTP cyclohydrolase I gene transcription within nigrostriatal dopamine neurons
G Kapatos - IUBMB life, 2013 - Wiley Online Library
Abstract Within the brain, the reduced pteridine cofactor 6R‐l‐erythro‐5, 6, 7, 8‐
tetrahydrobiopterin (BH4) is absolutely required for the synthesis of the monoamine (MA) …
tetrahydrobiopterin (BH4) is absolutely required for the synthesis of the monoamine (MA) …
Genetic and pharmacological correction of aberrant dopamine synthesis using patient iPSCs with BH4 metabolism disorders
T Ishikawa, K Imamura, T Kondo… - Human molecular …, 2016 - academic.oup.com
Dopamine (DA) is a neurotransmitter in the brain, playing a central role in several disease
conditions, including tetrahydrobiopterin (BH4) metabolism disorders and Parkinson's …
conditions, including tetrahydrobiopterin (BH4) metabolism disorders and Parkinson's …
Advanced research on dopamine signaling to develop drugs for the treatment of mental disorders: regulation of dopaminergic neural transmission by tyrosine …
C Sumi-Ichinose, H Ichinose, K Ikemoto… - Journal of …, 2010 - Elsevier
Abstract 5R-l-Erythro-5, 6, 7, 8-tetrahydrobiopterin (BH 4) is an essential cofactor for tyrosine
hydroxylase (TH). Recently, a type of dopa-responsive dystonia (DRD)(DYT5, Segawa's …
hydroxylase (TH). Recently, a type of dopa-responsive dystonia (DRD)(DYT5, Segawa's …
Tetrahydrobiopterin (BH4) treatment stabilizes tyrosine hydroxylase: Rescue of tyrosine hydroxylase deficiency phenotypes in human neurons and in a knock‐in …
K Jung‐kc, A Tristán‐Noguero… - Journal of Inherited …, 2024 - Wiley Online Library
Proteostatic regulation of tyrosine hydroxylase (TH), the rate‐limiting enzyme in dopamine
biosynthesis, is crucial for maintaining proper brain neurotransmitter homeostasis. Variants …
biosynthesis, is crucial for maintaining proper brain neurotransmitter homeostasis. Variants …
GCH1 deficiency activates brain innate immune response and impairs tyrosine hydroxylase homeostasis
H Larbalestier, M Keatinge, L Watson… - Journal of …, 2022 - Soc Neuroscience
The Parkinson's disease (PD) risk gene GTP cyclohydrolase 1 (GCH1) catalyzes the rate-
limiting step in tetrahydrobiopterin (BH4) synthesis, an essential cofactor in the synthesis of …
limiting step in tetrahydrobiopterin (BH4) synthesis, an essential cofactor in the synthesis of …
Genetically rescued tetrahydrobiopterin‐depleted mice survive with hyperphenylalaninemia and region‐specific monoaminergic abnormalities
C Sumi‐Ichinose, F Urano, A Shimomura… - Journal of …, 2005 - Wiley Online Library
One of the possibly mutated genes in DOPA‐responsive dystonia (DRD, Segawa's disease)
is the gene encoding GTP cyclohydrolase I, which is the rate‐limiting enzyme for …
is the gene encoding GTP cyclohydrolase I, which is the rate‐limiting enzyme for …
Tetrahydrobioterin (BH4) pathway: from metabolism to neuropsychiatry
H Fanet, L Capuron, N Castanon… - Current …, 2021 - ingentaconnect.com
Tetrahydrobipterin (BH4) is a pivotal enzymatic cofactor required for the synthesis of
serotonin, dopamine and nitric oxide. BH4 is essential for numerous physiological …
serotonin, dopamine and nitric oxide. BH4 is essential for numerous physiological …