Rat GTP cyclohydrolase 1, GCH1 ELISA Kit | MyBioSource MBS2602665 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Rat GTP cyclohydrolase 1, GCH1 ELISA Kit

catalog :

MBS2602665

quantity :

48-Strip-Wells

price :

290 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS2602665

products type :

ELISA Kit

products full name :

Rat GTP cyclohydrolase 1, GCH1 ELISA Kit

products short name :

GTP cyclohydrolase 1, GCH1

other names :

GTP cyclohydrolase 1; GTP cyclohydrolase 1; GTP cyclohydrolase 1; GTP-CH-I; GTP cyclohydrolase I; GTP cyclohydrolase 1; GTP cyclohydrolase I; GTP-CH-I

products gene name :

GCH1

other gene names :

Gch1; Gch1; Gch; Gch; GTP-CH-I

uniprot entry name :

GCH1_RAT

reactivity :

Rat

specificity :

No cross-reaction with other factors.

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Samples: Rat serum, plasma or Cell Culture Supernatant and organizations in the natural and recombinant GCH1 concentration. Assay Type: Sandwich. Detection Range: 10 ng/ml-0.156 ng/ml. Sensitivity: 0.05ng/ml.

other info2 :

Intra-assay Precision: <= 8%. Inter-assay Precision: <= 12%

products description :

Priciple of the assay: This experiment use double-sandwich elisa technique and the ELISA Kit provided is typical. The pre-coated antibody is Rat GCH1 monoclonal antibody and the detecting antibody is polyclonal antibody with biotin labeled. Samples and biotin labeling antibody are added into ELISA plate wells and washed out with PBS or TBS. Then Avidin-peroxidase conjugates are added to ELISA wells in order; Use TMB substrate for coloring after reactant thoroughly washed out by PBS or TBS. TMB turns into blue in peroxidase catalytic and finally turns into yellow under the action of acid. The color depth and the testing factors in samples are positively correlated.

ncbi gi num :

13242245

ncbi acc num :

NP_077332.1

ncbi gb acc num :

NM_024356.1

uniprot acc num :

P22288

ncbi mol weight :

27,057 Da

ncbi pathways :

Folate Biosynthesis Pathway (83410); Folate Biosynthesis Pathway (404)

ncbi summary :

an enzyme for tetrahydrobiopterin (BH(4)) synthesis; may mediate cell death of striatal neuronal and non-neuronal cells [RGD, Feb 2006]

uniprot summary :

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: Cofactor and Vitamin Metabolism - folate biosynthesis; EC 3.5.4.16; Hydrolase. Cellular Component: nucleoplasm; nuclear membrane; protein complex; cytoplasm; cytoplasmic vesicle; nucleus; cytosol. Molecular Function: GTP cyclohydrolase I activity; protein binding; protein homodimerization activity; GTP binding; zinc ion binding; GTP-dependent protein binding; calcium ion binding; coenzyme binding. Biological Process: tetrahydrobiopterin biosynthetic process; regulation of lung blood pressure; protein heterooligomerization; metabolic process; positive regulation of nitric-oxide synthase activity; dihydrobiopterin metabolic process; response to lipopolysaccharide; response to pain; dopamine biosynthetic process; pteridine and derivative biosynthetic process; vasodilation; neuromuscular process controlling posture; tetrahydrofolate biosynthetic process; regulation of blood pressure; protein complex assembly; negative regulation of blood pressure; protein homooligomerization

size1 :

48-Strip-Wells

price1 :

290 USD

size2 :

96-Strip-Wells

price2 :

450

more info or order :

MyBioSource product webpage