Recombinant Anti-GCH1 antibody [EPR24155-33] (ab259882)
![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)](https://www.abcam.com/ps/products/259/ab259882/Images/ab259882-1-anti-gch1-antibody-epr2415533-immunohistochemistry-rat-brain.jpg "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)")
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR24155-33] to GCH1
- Suitable for: IHC-P, Flow Cyt (Intra)
- Reacts with: Rat
Related conjugates and formulations
Overview
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Product name
Anti-GCH1 antibody [EPR24155-33]
See all GCH1 primary antibodies -
Description
Rabbit monoclonal [EPR24155-33] to GCH1 -
Host species
Rabbit -
Tested applications
Suitable for: IHC-P, Flow Cyt (Intra)more details
Unsuitable for: ICC/IF,IHC-Fr,IP or WB -
Species reactivity
Reacts with: Rat -
Immunogen
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- IHC-P: Rat brain and stomach tissues. Flow Cyt (intra): Rat primary neuron and glia cell.
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General notes
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
Preservative: 0.01% Sodium azide
Constituents: 59.94% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA -
Concentration information loading... -
Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
EPR24155-33 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Compatible Secondaries
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Isotype control
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab259882 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| IHC-P |
1/500.
|
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| Flow Cyt (Intra) |
1/500.
|
| Notes |
|---|
|
IHC-P
1/500. |
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Flow Cyt (Intra)
1/500. |
Target
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Function
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. -
Tissue specificity
In epidermis, expressed predominantly in basal undifferentiated keratinocytes and in some but not all melanocytes (at protein level). -
Pathway
Cofactor biosynthesis; 7,8-dihydroneopterin triphosphate biosynthesis; 7,8-dihydroneopterin triphosphate from GTP: step 1/1. -
Involvement in disease
Defects in GCH1 are the cause of GTP cyclohydrolase 1 deficiency (GCH1D) [MIM:233910]; 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) [MIM:128230]; 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. -
Sequence similarities
Belongs to the GTP cyclohydrolase I family. -
Post-translational
modificationsPhosphorylated by casein kinase II at Ser-81 in HAECs during oscillatory shear stress; phosphorylation at Ser-81 results in increased enzyme activity. -
Cellular localization
Cytoplasm. Nucleus. - Information by UniProt
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Database links
- Entrez Gene: 29244 Rat
- SwissProt: P22288 Rat
- Unigene: 28195 Rat
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Alternative names
- dystonia 14 antibody
- DYT 5 antibody
- DYT14 antibody
see all
Images
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)
Immunohistochemical analysis of paraffin-embedded Rat brain tissue labelling GCH1 with ab259882 at 1/500 (1.278 ug/ml) dilution followed by a ready to use Rabbit specific IHC polymer detection kit HRP/DAB (ab209101). Positive staining on ventral tegmental area of rat brain. The section was incubated with ab259882 for 30 mins at room temperature.The immunostaining was performed on a Leica Biosystems BOND RX instrument Counterstained with Hematoxylin.
Secondary antibody only control: Secondary antibody is a ready to use Rabbit specific IHC polymer detection kit HRP/DAB (ab209101).
Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0, epitope retrieval solution2) for 20 mins.
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![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)](https://www.abcam.com/ps/products/259/ab259882/Images/ab259882-2-anti-gch1-antibody-epr2415533-immunohistochemistry-rat-brain1.jpg)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)Immunohistochemical analysis of paraffin-embedded Rat brain tissue labelling GCH1 with ab259882 at 1/500 (1.278 ug/ml) dilution followed by a ready to use Rabbit specific IHC polymer detection kit HRP/DAB (ab209101). Positive staining on ventral tegmental area of rat brain. The section was incubated with ab259882 for 30 mins at room temperature.The immunostaining was performed on a Leica Biosystems BOND RX instrument Counterstained with Hematoxylin.
Secondary antibody only control: Secondary antibody is a ready to use Rabbit specific IHC polymer detection kit HRP/DAB (ab209101).
Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0, epitope retrieval solution2) for 20 mins.
-
![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)](https://www.abcam.com/ps/products/259/ab259882/Images/ab259882-3-anti-gch1-antibody-epr2415533-immunohistochemistry-rat-stomach.jpg)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GCH1 antibody [EPR24155-33] (ab259882)Immunohistochemical analysis of paraffin-embedded Rat stomach tissue labelling GCH1 with ab259882 at 1/500 (1.278 ug/ml) dilution followed by a ready to use Rabbit specific IHC polymer detection kit HRP/DAB (ab209101). Positive staining on rat stomach. The section was incubated with ab259882 for 30 mins at room temperature.The immunostaining was performed on a Leica Biosystems BOND RX instrument Counterstained with Hematoxylin.
Secondary antibody only control: Secondary antibody is a ready to use Rabbit specific IHC polymer detection kit HRP/DAB (ab209101).
Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0, epitope retrieval solution2) for 20 mins.
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![Flow Cytometry (Intracellular) - Anti-GCH1 antibody [EPR24155-33] (ab259882)](https://www.abcam.com/ps/products/259/ab259882/Images/ab259882-4-anti-gch1-antibody-epr2415533-flow-cytometry-rat-primary-neuron-and-glia-cells.jpg)
Flow Cytometry (Intracellular) - Anti-GCH1 antibody [EPR24155-33] (ab259882)Intracellular flow cytometric analysis of 4% paraformaldehyde fixed 90% methanol permeabilized Rat primary neuron and glia cells cells labelling GCH1 with ab259882 at 1/500 dilution (0.1ug). Right (Red) compared with a Rabbit monoclonal IgG (ab172730) / Left isotype control. A Goat anti rabbit IgG (Alexa Fluor® 488, ab150077) at 1/2000 dilution was used as the secondary antibody.
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![Anti-GCH1 antibody [EPR24155-33] (ab259882)](https://www.abcam.com/ps/products/259/ab259882/Images/ab259882-5-Benefits-of-Recombinant-antibodies.jpg)
Anti-GCH1 antibody [EPR24155-33] (ab259882)
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
Certificate of Compliance
References (0)
ab259882 has not yet been referenced specifically in any publications.