1-832-868-1888
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Catalog Number: |
33000 |
| other_names: | DHPR; PKU2; SDR33C1; |
Amount: |
100μg |
| calculated_mw: | |
| host_species: | Rabbit |
Price: |
$319 |
Swiss-Prot No: |
Swiss-Prot:P09417 |
Form of Antibody: |
Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
Storage/Stability: |
|
Immunogen: |
Recombinant protein of human QDPR. |
Purification: |
Antibodies were purified by affinity purification using immunogen. |
Specificity/Sensitivity: |
The antibody detects endogenous level of total QDPR protein. |
Applications: |
WB,IF |
Background: |
This gene encodes the enzyme dihydropteridine reductase, which catalyzes the NADH-mediated reduction of quinonoid dihydrobiopterin. This enzyme is an essential component of the pterin-dependent aromatic amino acid hydroxylating systems. Mutations in this gene resulting in QDPR deficiency include aberrant splicing, amino acid substitutions, insertions, or premature terminations. Dihydropteridine reductase deficiency presents as atypical phenylketonuria due to insufficient production of biopterin, a cofactor for phenylalanine hydroxylase. |
References: |
|
appl_detail: |
WB 1:500 - 1:2000 |
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