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DHPR, alpha 2, Monoclonal Antibody

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产品名称: DHPR, alpha 2, Monoclonal Antibody
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简单介绍

DHPR, alpha 2, Monoclonal Antibody


DHPR, alpha 2, Monoclonal Antibody  的详细介绍
Product Name

DHPR, alpha 2 (Qdpr), Monoclonal Antibody

Full Product Name

DHPR, alpha 2 (Dihydropyridine Receptor, CACNA1S)

Product Synonym Names
Anti -DHPR, alpha 2 (Dihydropyridine Receptor, CACNA1S)
Product Gene Name

anti-Qdpr antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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Chromosome Location
Chromosome: 5; NC_000071.6 (45434032..45450229, complement). Location: 5 B3; 5 24.9 cM
3D Structure
ModBase 3D Structure for Q8BVI4
Clonality
Monoclonal
Isotype
IgG2a
Clone Number
6D357
Host
Mouse
Species Reactivity
Guinea Pig, Human, Mouse, Rabbit, Rat
Specificity
Recognizes the rabbit alpha-2 subunit of DHP receptor.
Purity/Purification
Ascites
Ascites
Form/Format
Supplied as a liquid in 0.05% sodium azide.
Immunogen
Purified rabbit DHP receptor (dihydropyridine receptor)
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. 
Other Notes
Small volumes of anti-Qdpr antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-Qdpr antibody
Voltage-sensitive calcium channels mediate the entry of calcium into many types of excitable cells and thus play a key role in neurotransmitter release and excitation-contraction (E-C) coupling. The 1,4-dihydropyridines (DHPs) are synthetic organic compounds which can be used to identify the L-type calcium channels that are found in all types of vertebrate muscle, neuronal and neuroendocrine cells. The DHP receptor is part of the L-type calcium channel complex and is thought to be the voltage sensor in E-C coupling. The purified DHP receptor isolated from triads is composed of at least four subunits. The alpha-1 subunit contains the for the DHPs and shows high sequence homology to the voltage gated sodium channel. The alpha-2 subunit is a large glycoprotein associated with the DHP receptor which was first described in skeletal muscle and is also found in high concentrations in other excitable tissues such as cardiac muscle and brain and in low concentrations in most other tissues studied. The other two subunits that co-purify with the DHP receptor are termed beta and gamma.
Product Categories/Family for anti-Qdpr antibody
Antibodies; Abs to Receptors
Applications Tested/Suitable for anti-Qdpr antibody
Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC)
Application Notes for anti-Qdpr antibody
Suitable for use in Immunohistochemistry, Immunocytochemistry and Western Blot.
Dilution: Immunohistochemistry (Paraffin, frozen): 1:500. Staining of DHP in rabbit skeletal muscle results in double rows of discrete punctate staining representing pairs of triads on the opposing sides of the Z-lines.
Immunocytochemistry: 1:250
Western Blot: 1:500. Detects a band of ~220kD (non-reducing conditions) or ~143kD (reducing conditions).
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NCBI/Uniprot data below describe general gene information for Qdpr. It may not necessarily be applicable to this product.
NCBI GI #
21312520
NCBI GeneID
110391
NCBI Accession #
NP_077198.1 [Other Products]
NCBI GenBank Nucleotide #
NM_024236.2 [Other Products]
UniProt Primary Accession #
Q8BVI4 [Other Products]
UniProt Secondary Accession #
Q3TT09; Q9D0K4[Other Products]
UniProt Related Accession #
Q8BVI4[Other Products]
Molecular Weight
25,570 Da[Similar Products]
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NCBI Official Full Name
dihydropteridine reductase
NCBI Official Synonym Full Names
quinoid dihydropteridine reductase
NCBI Official Symbol
Qdpr  [Similar Products]
NCBI Official Synonym Symbols
Dhpr; PKU2; D5Ertd371e; 2610008L04Rik
  [Similar Products]
NCBI Protein Information
dihydropteridine reductase; HDHPR
UniProt Protein Name
Dihydropteridine reductase
UniProt Synonym Protein Names
HDHPR; Quinoid dihydropteridine reductase
Protein Family
Dihydropteridine reductase
UniProt Gene Name
Qdpr  [Similar Products]
UniProt Synonym Gene Names
Dhpr  [Similar Products]
UniProt Entry Name
DHPR_MOUSE
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UniProt Comments for Qdpr
QDPR: The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases. Defects in QDPR are the cause of BH4-deficient hyperphenylalaninemia type C (HPABH4C); also called dihydropteridine reductase deficiency (DHPR deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to DHPR deficiency or quinoid dihydropteridine reductase deficiency (QDPR deficiency). HPABH4C is a rare autosomal recessive disorder characterized by hyperphenylalaninemia and severe neurologic symptoms (malignant hyperphenylalaninemia) including axial hypotonia and truncal hypertonia, abnormal thermogenesis, and microcephaly. These signs are attributable to depletion of the neurotransmitters dopamine and serotonin, whose syntheses are controlled by tryptophan and tyrosine hydroxylases that use BH-4 as cofactor. These patients do not respond to phenylalanine- restricted diet. HPABH4C is lethal if untreated. Belongs to the short-chain dehydrogenases/reductases (SDR) family.

Protein type: EC 1.5.1.34; Oxidoreductase; Cofactor and Vitamin Metabolism - folate biosynthesis

Cellular Component: neuron projection; mitochondrion; cytoplasm; cytosol

Molecular Function: protein homodimerization activity; 6,7-dihydropteridine reductase activity; oxidoreductase activity

Biological Process: tetrahydrobiopterin biosynthetic process; metabolic process
Research Articles on Qdpr
1. JP1 and JP2 can facilitate the assembly of DHPR with other proteins of the excitation-contraction coupling machinery
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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