Metabolism Antibodies 2
Anti-QDPR Antibody (CAB5733)
- SKU:
- CAB5733
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-QDPR Antibody |
| Antibody SKU: | CAB5733 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-244 of human QDPR (NP_000311.2). |
| Application: | WB IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:100 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | Mouse liver, Mouse kidney, Mouse brain, Rat liver, HL-60, BT-474, SW620 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-244 of human QDPR (NP_000311.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MAAA AAAG EARR VLVY GGRG ALGS RCVQ AFRA RNWW VASV DVVE NEEA SASI IVKM TDSF TEQA DQVT AEVG KLLG EEKV DAIL CVAG GWAG GNAK SKSL FKNC DLMW KQSI WTST ISSH LATK HLKE GGLL TLAG AKAA LDGT PGMI GYGM AKGA VHQL CQSL AGKN SGMP PGAA AIAV LPVT LDTP MNRK SMPE ADFS SWTP LEFL VETF HDWI TGKN RPSS GSLI QVVT TEGR TELT PAYF |
| Gene ID: | 5860 |
| Uniprot: | P09417 |
| Cellular Location: | |
| Calculated MW: | 22kDa/25kDa |
| Observed MW: | 26kDa |
| Synonyms: | QDPR, DHPR, PKU2, SDR33C1 |
| 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. |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Oxidoreductase; Cofactor and Vitamin Metabolism - folate biosynthesis; EC 1.5.1.34 Chromosomal Location of Human Ortholog: 4p15.31 Cellular Component: cytoplasm; cytosol Molecular Function:6,7-dihydropteridine reductase activity; electron carrier activity Biological Process: amino acid metabolic process; dihydrobiopterin metabolic process; L-phenylalanine catabolic process Disease: Hyperphenylalaninemia, Bh4-deficient, C |
| NCBI Summary: | 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. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P09417 |
| NCBI GenInfo Identifier: | 118572639 |
| NCBI Gene ID: | 5860 |
| NCBI Accession: | P09417.2 |
| UniProt Secondary Accession: | P09417,Q53F52, Q9H3M5, A8K158, B3KW71, |
| UniProt Related Accession: | P09417 |
| Molecular Weight: | 22,408 Da |
| NCBI Full Name: | Dihydropteridine reductase |
| NCBI Synonym Full Names: | quinoid dihydropteridine reductase |
| NCBI Official Symbol: | QDPR |
| NCBI Official Synonym Symbols: | DHPR; PKU2; SDR33C1 |
| NCBI Protein Information: | dihydropteridine reductase |
| UniProt Protein Name: | Dihydropteridine reductase |
| UniProt Synonym Protein Names: | HDHPR; Quinoid dihydropteridine reductase; Short chain dehydrogenase/reductase family 33C member 1 |
| Protein Family: | Dihydropteridine reductase |
| UniProt Gene Name: | QDPR |
| UniProt Entry Name: | DHPR_HUMAN |
 | Western blot analysis of extracts of various cell lines, using QDPR antibody (CAB5733) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
 | Western blot analysis of extracts of various cell lines, using QDPR antibody (CAB5733) at 1:3000 dilution._Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution._Lysates/proteins: 25ug per lane._Blocking buffer: 3% nonfat dry milk in TBST._Detection: ECL Enhanced Kit (RM00021)._Exposure time: 90s. |
 | Immunofluorescence analysis of U2OS cells using QDPR antibody (CAB5733). Blue: DAPI for nuclear staining. |
View AllClose
Related Products
Human QDPR Recombinant Protein (RPPB2236)
Enzymes Recombinant Proteins
system_update_altDatasheetsystem_update_altMSDS
