2024年5月1日 · Here, we showed that ionizing radiation disrupted the structural integrity of BH4 and downregulated GTP cyclohydrolase I (GCH1), which is the rate-limiting enzyme in BH4 biosynthesis, resulting...

2015年6月17日 · We therefore generated inducible sensory neuron-specific Gch1 KO mice to show that blocking BH4 production is sufficient to partially reverse established hypersensitivity, indicating that BH4 is involved in maintaining the pain phenotype and, as a consequence, that targeting this pathway in injured peripheral neurons may have therapeutic utility.

2024年12月25日 · GCH1 (GTP Cyclohydrolase 1) is a Protein Coding gene. Diseases associated with GCH1 include Hyperphenylalaninemia, Bh4-Deficient, B and Dystonia, Dopa-Responsive. Among its related pathways are erythro-tetrahydrobiopterin biosynthesis II and …

2024年3月15日 · In this study, we tested the efficacy of dopa and BH4 therapies by using a novel GTPCH deficiency mouse model, Gch1KI/KI, which manifested infancy-onset motor deficits and growth retardation similar to the patients. First, dopa supplementation supported Gch1KI/KI mouse survival to adulthood, but residual motor deficits and dwarfism remained.

2023年5月15日 · The cmGch1 KO mouse provides a new model to understand the cell‐ and tissue‐specific relationships between Gch1 expression, BH4 synthesis and BH4 recycling or uptake.

2020年8月10日 · Guide resistant GCH1 (P30793–1) and SPR (P35270–1) cDNA was designed and codon-optimized. The truncated isoform, GCH1–3 (P30793–3), was amplified using Phusion High Fidelity PCR (NEB).

2015年6月17日 · We therefore generated inducible sensory neuron-specific Gch1 KO mice to show that blocking BH4 production is sufficient to partially reverse established hypersensitivity, indicating that BH4 is involved in maintaining the pain phenotype and, as a consequence, that targeting this pathway in injured peripheral neurons may have therapeutic utility.

2024年9月19日 · Gpx4 KO HapT1 cells exhibited a much faster accumulation of lipid peroxide than parental (wild type; WT) HapT1 cells, whereas both Gpx4 KO and WT HapT1 cells exhibited very low levels of lipid...

2024年5月7日 · BH4是一氧化氮合（NOS）的重要辅助因子，由GTP经过限速酶GTP环化水解酶1（GTP cyclohydrolase 1，GCH1）等催化产生。 BH4在抗肿瘤方面发挥着重要的作用，2018年Natuer报道通过GCH1过表达增强BH4水平可增强表达CD4-和CD8-的T细胞的应答，增强其体内抗肿瘤活性[1]。 该研究团队前期发现Nrf2/GCH1轴参与辐射诱导的ROS产生从而参与放射性皮肤损伤的进展[2]，该报道提出辐射诱导的ROS产生被认为是NOS解偶联的结果[2]。 多项研究表 …

2023年5月15日 · The cmGch1 KO mouse provides a new model to understand the cell- and tissue-specific relationships between Gch1 expression, BH4 synthesis and BH4 recycling or uptake. Gch1 mRNA expression was almost completely abolished in isolated cardiomyocytes from cmGch1 KO mice, whereas BH4 levels were maintained at approximately 40% of …