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PH / PAH
phenylalanine hydroxylase
PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.
| Gene Name: | phenylalanine hydroxylase |
| Synonyms: | PAH, Phe-4-monooxygenase, PKU1, PH, Phenylalanine 4-monooxygenase, Phe-4- monooxygenase, Phenylalanine hydroxylase, Phenylalanine-4-hydroxylase, PKU |
| Target Sequences: | NM_000277 NP_000268.1 P00439 |
Publications (1)
1
CRISPR/Cas9 generated knockout mice lacking phenylalanine hydroxylase protein as a novel preclinical model for human phenylketonuria10<sup> Genomic Medicine Unit, Sanofi, 49 New York Avenue, Framingham, MA, 01701, USA. Sirkka.Kyostio-Moore@sanofi.com. Kuldeep Singh , Cathleen S Cornell, Robert Jackson, Mostafa Kabiri, Michael Phipps, Mitul Desai, Robert Fogle, Xiaoyou Ying, Gulbenk Anarat-Cappillino, Sarah Geller, Jennifer Johnson, Errin Roberts, Katie Malley, Tim Devlin, Matthew DeRiso, Patricia Berthelette, Yao V Zhang, Susan Ryan, Srinivas Rao, Beth L Thurberg, Dinesh S Bangari, Sirkka Kyostio-Moore. Scientific reports. 2021 Mar;11:7254. [Full Text Article]
[PubMed:33790381]
[PMC:PMC8012645]
Related Antibodies: LS-C344145.
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Products
Proteins
(8)
Type
Over-Expression Lysate
(2)
Recombinant
(6)
Target
PH / PAH
(8)
Publications
No
(8)
Tag
6His-SUMO, N-terminus
(1)
GST
(1)
His
(3)
Myc-DDK (Flag)
(3)
Species
Human
(7)
Source
293T Cells
(1)
Baculovirus
(1)
E. coli
(3)
HEK 293 Cells
(2)
Wheat Germ Extract
(1)
Purification
Purified
(3)
Low Endotoxin Level
Low endotoxin level
(1)
Wheat Germ Extract
GST
78.2 kDa
10 µg/$479; 25 µg/$670
HEK 293 Cells
Myc-DDK (Flag)
51.7 kDa
20 µg/$1,107
293T Cells
Myc-DDK (Flag)
51.7 kDa
20 µg/$150
HEK 293 Cells
Myc-DDK (Flag)
51.7 kDa
100 µg/$494
E. coli
6His-SUMO, N-terminus
1 mg/$1,355; 100 µg/$420; 20 µg/$323
Baculovirus
His
Less than 1.0 EU/µg protein (determined by LAL method).
54 kDa
50 µg/$854
E. coli
His
27.9 kDa
10 µg/$291; 50 µg/$502; 100 µg/$802; 200 µg/$1,002; 1 mg/$2,144; 500 µg/$1,668; 5 mg/$3,501; 2 mg/$2,334
E. coli
His
26.9 kDa
10 µg/$291; 50 µg/$502; 100 µg/$802; 200 µg/$1,002; 1 mg/$2,144; 500 µg/$1,668; 5 mg/$3,501; 2 mg/$2,334
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