1TDW

Crystal structure of double truncated human phenylalanine hydroxylase BH4-responsive PKU mutant A313T.

Summary for 1TDW

Related1PAH
DescriptorPhenylalanine-4-hydroxylase, FE (III) ION (3 entities in total)
Functional Keywordsphenylalanine catabolism, oxidoreductase
Biological sourceHomo sapiens (human)
Total number of polymer chains1
Total molecular weight35786.39
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: 2004-05-24, release date: 2004-11-30, Last modification date: 2017-10-11)
Primary citation
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.
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
PubMed: 15557004 (PDB entries with the same primary citation)
DOI: 10.1073/pnas.0407256101
MImport into Mendeley
Experimental method
X-RAY DIFFRACTION (2.1 Å)
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Structure validation

RfreeClashscoreRamachandran outliersSidechain outliersRSRZ outliers 0.23780 2.6% 3.2%MetricValuePercentile RanksWorseBetterPercentile relative to all X-ray structuresPercentile relative to X-ray structures of similar resolution
Download full validation reportDownload
171588
PDB entries from 2020-11-25