QDPR Rabbit pAb

产品详情
实验流程

Product Information
Application Applications Legend: E=ELISA WB=Western Blotting IHC=Immunohistochemistry IHC-P=Immunohistochemistry (Paraffin) IP=Immunoprecipitation IF=Immunofluorescence IC=Immunochemistry ICC=Immunocytochemistry FC=Flow Cytometry DB=Dot Blot	IHC-P, IHC-F, IF
Primary Accession	P09417
Other Accession	P09417
Host	Rabbit
Clonality	Polyclonal
Calculated MW	25790 Da
Physical State	Liquid
Immunogen	KLH conjugated synthetic peptide derived from human QDPR
Epitope Specificity	141-244/244
Isotype	IgG
Purity	affinity purified by Protein A
Buffer	0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY	Belongs to the short-chain dehydrogenases/reductases (SDR) family.
SUBUNIT	Homodimer.
DISEASE	Defects in QDPR are the cause of BH4-deficient hyperphenylalaninemia type C (HPABH4C) [MIM:261630]; 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.
Important Note	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions	6,7 dihydropteridine reductase; DHPR; DHPR_HUMAN; Dihydropteridine reductase; HDHPR; HPR; PKU2; Qdpr; Quinoid dihydropteridine reductase; SDR33C1; Short chain dehydrogenase/reductase family 33C, member 1.

Additional Information
Gene ID	5860
Other Names	DHPR; HDHPR; PKU2; SDR33C1; 2610008L04Rik; D5Ertd371e; DHPR_HUMAN; QDPR; Quinoid dihydropteridine reductase; Short chain dehydrogenase/reductase family 33C member 1; 1.5.1.34; DHPR_MOUSE; DHPR_RAT; 6, 7-dihydropteridine reductase; short chain dehydrogenase/reductase family 33C, member 1
Dilution	IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.