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1TG2

Crystal structure of phenylalanine hydroxylase A313T mutant with 7,8-dihydrobiopterin bound

Summary for 1TG2
Entry DOI10.2210/pdb1tg2/pdb
Related1LRM 1PAH 1TDW
DescriptorPhenylalanine-4-hydroxylase, FE (III) ION, 2-AMINO-6-(1,2-DIHYDROXY-PROPYL)-7,8-DIHYDRO-6H-PTERIDIN-4-ONE, ... (4 entities in total)
Functional Keywordsphenylalanine hydroxylase phenylketonuria mutant a313t in complex with cofactor analogue 7, 8-dihydrobiopterin, oxidoreductase
Biological sourceHomo sapiens (human)
Total number of polymer chains1
Total formula weight36025.62
Authors
Erlandsen, H.,Pey, A.L.,Gamez, A.,Perez, B.,Desviat, L.R.,Aguado, C.,Koch, R.,Surendran, S.,Tyring, S.,Matalon, R.,Scriver, C.R.,Ugarte, M.,Martinez, A.,Stevens, R.C. (deposition date: 2004-05-28, release date: 2004-11-30, Last modification date: 2023-08-23)
Primary citationErlandsen, H.,Pey, A.L.,Gamez, A.,Perez, B.,Desviat, L.R.,Aguado, C.,Koch, R.,Surendran, S.,Tyring, S.,Matalon, R.,Scriver, C.R.,Ugarte, M.,Martinez, A.,Stevens, R.C.
Correction of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations.
Proc.Natl.Acad.Sci.Usa, 101:16903-16908, 2004
Cited by
PubMed: 15557004
DOI: 10.1073/pnas.0407256101
PDB entries with the same primary citation
Experimental method
X-RAY DIFFRACTION (2.2 Å)
Structure validation

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