GTP-CH-1 polyclonal, anti-human, mouse, rat
€378.00
In stock
SKU
250680
Catalog Nr.: 250680
Size: 0.1 mg
Isotype: Rabbit Ig
Applications: E, WB, IHC
Size: 0.1 mg
Isotype: Rabbit Ig
Applications: E, WB, IHC
Protein Family: Enzymes
Pathway and Disease: Cofactor and Vitamin Metabolism
Description:
GTP cyclohydrolase 1 (GTP-CH-1 or GCH-1) catalyzes the conversion of GTP to D-erythro-7,8-dihydroneopterin triphosphate, the first and rate-limiting step in tetrahydrobiopterin (BH4) biosynthesis. The final product (BH4) is an essential co-factor for three aromatic amino acid monooxygenases (F, Y, and W). Isoform GCH-1 is the functional enzyme. GTP-CH-1 positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVEC). GTP-CH-1 deficiency is a cause of malignant hyperphenylalaninemia due to tetrahydrobiopterin deficiency. It is also responsible for defective neurotransmission due to depletion of the neurotransmitters dopamine and serotonin. GTP-CH-1 (DYT5) is a cause for dystonia 5, a DOPA-responsive dystonia, defined by sustained involuntary muscle contractions.
Alternate Names: GCH-1, GTP cyclohydrolase I, GTP-CH-1, GTP cyclohydrolase 1, GTP-CH-I, GCH1, DYT5, GCH
Application Notes: E: 1:500-1:1,000; WB: 1:200-1:500; IHC: 1:200-1:500
Accession No.: P30793
Antigen: KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human GTP-CH-1.
Format: Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 0.1 mg/ml BSA, 25% glycerol with 0.09% sodium azide. Antibody was purified by Protein-A affinity chromatography.
Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Product Citations:
[1] Blecharz KG et al. 2014. Biol Cell. 106(7): 219-35. PMID# 24749543.
[2] Meng C et al. 2013. Neurological Sciences. 10.1007/s10072-013-1353-5. PMID# 23515624.
Pathway and Disease: Cofactor and Vitamin Metabolism
Description:
GTP cyclohydrolase 1 (GTP-CH-1 or GCH-1) catalyzes the conversion of GTP to D-erythro-7,8-dihydroneopterin triphosphate, the first and rate-limiting step in tetrahydrobiopterin (BH4) biosynthesis. The final product (BH4) is an essential co-factor for three aromatic amino acid monooxygenases (F, Y, and W). Isoform GCH-1 is the functional enzyme. GTP-CH-1 positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVEC). GTP-CH-1 deficiency is a cause of malignant hyperphenylalaninemia due to tetrahydrobiopterin deficiency. It is also responsible for defective neurotransmission due to depletion of the neurotransmitters dopamine and serotonin. GTP-CH-1 (DYT5) is a cause for dystonia 5, a DOPA-responsive dystonia, defined by sustained involuntary muscle contractions.
Alternate Names: GCH-1, GTP cyclohydrolase I, GTP-CH-1, GTP cyclohydrolase 1, GTP-CH-I, GCH1, DYT5, GCH
Application Notes: E: 1:500-1:1,000; WB: 1:200-1:500; IHC: 1:200-1:500
Accession No.: P30793
Antigen: KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human GTP-CH-1.
Format: Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 0.1 mg/ml BSA, 25% glycerol with 0.09% sodium azide. Antibody was purified by Protein-A affinity chromatography.
Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Product Citations:
[1] Blecharz KG et al. 2014. Biol Cell. 106(7): 219-35. PMID# 24749543.
[2] Meng C et al. 2013. Neurological Sciences. 10.1007/s10072-013-1353-5. PMID# 23515624.
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