Rabbit anti-Mouse, Rat GTP cyclohydrolase 1 Polyclonal Antibody | anti-GTP antibody
GTP cyclohydrolase 1 Antibody FITC
ELISA: 1:20,000
Western Blot: 1:500-1:750
Positively regulates nitric oxide synthesis in endothelial cells. Invovled in dopamine synthesis and pain sensitivity and persistence.
NCBI and Uniprot Product Information
Uniprot Description
GCH1: Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown. Defects in GCH1 are the cause of GTP cyclohydrolase 1 deficiency (GCH1D); also known as atypical severe phenylketonuria due to GTP cyclohydrolase I deficiency;. GCH1D is one of the causes of malignant hyperphenylalaninemia due to tetrahydrobiopterin deficiency. It is also responsible for defective neurotransmission due to depletion of the neurotransmitters dopamine and serotonin. The principal symptoms include: psychomotor retardation, tonicity disorders, convulsions, drowsiness, irritability, abnormal movements, hyperthermia, hypersalivation, and difficulty swallowing. Some patients may present a phenotype of intermediate severity between severe hyperphenylalaninemia and mild dystonia type 5 (dystonia- parkinsonism with diurnal fluctuation). In this intermediate phenotype, there is marked motor delay, but no mental retardation and only minimal, if any, hyperphenylalaninemia. Defects in GCH1 are the cause of dystonia type 5 (DYT5); also known as progressive dystonia with diurnal fluctuation, autosomal dominant Segawa syndrome or dystonia- parkinsonism with diurnal fluctuation. DYT5 is a DOPA-responsive dystonia. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. DYT5 typically presents in childhood with walking problems due to dystonia of the lower limbs and worsening of the dystonia towards the evening. It is characterized by postural and motor disturbances showing marked diurnal fluctuation. Torsion of the trunk is unusual. Symptoms are alleviated after sleep and aggravated by fatigue and excercise. There is a favorable response to L-DOPA without side effects. Belongs to the GTP cyclohydrolase I family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.5.4.16; Cofactor and Vitamin Metabolism - folate biosynthesis; Hydrolase
Cellular Component: cytoplasm; cytoplasmic vesicle; cytosol; nuclear membrane; nucleoplasm; nucleus; protein complex
Molecular Function: calcium ion binding; catalytic activity; coenzyme binding; GTP binding; GTP cyclohydrolase I activity; GTP-dependent protein binding; hydrolase activity; metal ion binding; nucleotide binding; protein homodimerization activity; translation initiation factor binding; zinc ion binding
Biological Process: dihydrobiopterin metabolic process; dopamine biosynthetic process; metabolic process; negative regulation of blood pressure; neuromuscular process controlling posture; positive regulation of nitric-oxide synthase activity; protein complex assembly; protein heterooligomerization; protein homooligomerization; pteridine and derivative biosynthetic process; regulation of blood pressure; regulation of lung blood pressure; response to pain; tetrahydrobiopterin biosynthetic process; tetrahydrofolate biosynthetic process; vasodilation
Research Articles on GTP
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Product Notes
The GTP gch1 (Catalog #AAA541791) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GTP cyclohydrolase 1 Antibody FITC reacts with Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's GTP cyclohydrolase 1 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC), Immunoprecipitation (IP), Western Blot (WB). Dot Blot: 1:20,000 ELISA: 1:20,000 Western Blot: 1:500-1:750. Researchers should empirically determine the suitability of the GTP gch1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GTP cyclohydrolase 1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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