The structure of full-length human phenylalanine hydroxylase in complex with the cofactor and negative regulator tetrahydrobiopterin. Determined by X-ray diffraction at 3.18 Å resolution. Released 5 Jun 2019.
Explore 6HYC in 3D Show helices and sheets RCSB PDB PDBe
6HYC contains 98 α-helices and 68 β-strands across 4 chains. Residue ranges use author residue numbering, as in the PDB file, from PDBe. To see them in 3D, choose the Cartoon representation with Secondary structure coloring: helices, sheets and coils get different colors.
| Element | Residues | Length | Sheet |
|---|---|---|---|
| β-strand | 35-38 | 4 | 6 |
| β-strand | 39-42 | 4 | 7 |
| α-helix | 47-57 | 11 | |
| β-strand | 65-69 | 5 | 7 |
| β-strand | 76-81 | 6 | 7 |
| α-helix | 85-87 | 3 | |
| α-helix | 88-96 | 9 | |
| α-helix | 97-102 | 6 | |
| β-strand | 107-110 | 4 | 6 |
| α-helix | 117 | 1 | |
| β-strand | 118-119 | 2 | 6 |
| α-helix | 120 | 1 | |
| β-strand | 124 | 1 | 8 |
| α-helix | 127-129 | 3 | |
| α-helix | 152-167 | 16 | |
| α-helix | 173-176 | 4 | |
| α-helix | 181-201 | 21 | |
| β-strand | 202 | 1 | 9 |
| α-helix | 204-217 | 14 | |
| α-helix | 227-238 | 12 | |
| β-strand | 241-244 | 4 | 10 |
| α-helix | 251-259 | 9 | |
| β-strand | 262-265 | 4 | 10 |
| α-helix | 283-285 | 3 | |
| α-helix | 286-290 | 5 | |
| α-helix | 291-294 | 4 | |
| α-helix | 297-310 | 14 | |
| α-helix | 315-325 | 11 | |
| α-helix | 326-330 | 5 | |
| β-strand | 332-336 | 5 | 9 |
| β-strand | 339-342 | 4 | 9 |
| α-helix | 347-349 | 3 | |
| α-helix | 351-356 | 6 | |
| β-strand | 363-366 | 4 | 9 |
| α-helix | 369-372 | 4 | |
| β-strand | 385-389 | 5 | 9 |
| α-helix | 392-403 | 12 | |
| β-strand | 411-415 | 5 | 8 |
| β-strand | 420-424 | 5 | 8 |
| α-helix | 426-448 | 23 |
| Element | Residues | Length | Sheet |
|---|---|---|---|
| β-strand | 35-38 | 4 | 17 |
| β-strand | 39-42 | 4 | 18 |
| α-helix | 47-57 | 11 | |
| β-strand | 65-69 | 5 | 18 |
| β-strand | 76-81 | 6 | 18 |
| β-strand | 83 | 1 | 17 |
| α-helix | 85-87 | 3 | |
| α-helix | 88-96 | 9 | |
| α-helix | 97-102 | 6 | |
| β-strand | 107-110 | 4 | 17 |
| β-strand | 118-119 | 2 | 17 |
| α-helix | 120 | 1 | |
| β-strand | 124 | 1 | 19 |
| α-helix | 127-129 | 3 | |
| α-helix | 152-167 | 16 | |
| α-helix | 173-176 | 4 | |
| α-helix | 181-201 | 21 | |
| β-strand | 202 | 1 | 20 |
| α-helix | 204-213 | 10 | |
| α-helix | 214-218 | 5 | |
| α-helix | 224-226 | 3 | |
| α-helix | 227-238 | 12 | |
| β-strand | 241-244 | 4 | 21 |
| α-helix | 251-259 | 9 | |
| β-strand | 262-265 | 4 | 21 |
| α-helix | 283-285 | 3 | |
| α-helix | 286-290 | 5 | |
| α-helix | 291-295 | 5 | |
| α-helix | 297-310 | 14 | |
| α-helix | 315-325 | 11 | |
| α-helix | 326-330 | 5 | |
| β-strand | 332-336 | 5 | 20 |
| β-strand | 339-342 | 4 | 20 |
| α-helix | 351-357 | 7 | |
| β-strand | 363-366 | 4 | 20 |
| α-helix | 369-372 | 4 | |
| β-strand | 385-389 | 5 | 20 |
| α-helix | 392-403 | 12 | |
| β-strand | 411-415 | 5 | 19 |
| β-strand | 420-424 | 5 | 19 |
| α-helix | 426-447 | 22 |
| Element | Residues | Length | Sheet |
|---|---|---|---|
| β-strand | 35-38 | 4 | 11 |
| β-strand | 39-42 | 4 | 12 |
| α-helix | 47-57 | 11 | |
| β-strand | 65-69 | 5 | 12 |
| β-strand | 76-81 | 6 | 12 |
| β-strand | 83 | 1 | 11 |
| α-helix | 85-87 | 3 | |
| α-helix | 88-96 | 9 | |
| α-helix | 97-102 | 6 | |
| β-strand | 107-110 | 4 | 11 |
| β-strand | 118-119 | 2 | 11 |
| α-helix | 120 | 1 | |
| β-strand | 124 | 1 | 13 |
| α-helix | 127-129 | 3 | |
| β-strand | 135 | 1 | 14 |
| α-helix | 152-167 | 16 | |
| α-helix | 181-201 | 21 | |
| β-strand | 202 | 1 | 15 |
| α-helix | 204-207 | 4 | |
| α-helix | 209-213 | 5 | |
| α-helix | 214-218 | 5 | |
| α-helix | 227-238 | 12 | |
| β-strand | 241-244 | 4 | 16 |
| β-strand | 249 | 1 | 14 |
| α-helix | 251-259 | 9 | |
| β-strand | 262-265 | 4 | 16 |
| α-helix | 283-285 | 3 | |
| α-helix | 286-290 | 5 | |
| α-helix | 291-294 | 4 | |
| α-helix | 297-310 | 14 | |
| α-helix | 315-325 | 11 | |
| α-helix | 326-330 | 5 | |
| β-strand | 332-336 | 5 | 15 |
| β-strand | 339-342 | 4 | 15 |
| α-helix | 351-357 | 7 | |
| β-strand | 363-366 | 4 | 15 |
| α-helix | 369-372 | 4 | |
| β-strand | 385-389 | 5 | 15 |
| α-helix | 392-403 | 12 | |
| β-strand | 411-415 | 5 | 13 |
| β-strand | 420-424 | 5 | 13 |
| α-helix | 426-447 | 22 |
| Element | Residues | Length | Sheet |
|---|---|---|---|
| β-strand | 35-38 | 4 | 1 |
| β-strand | 39-42 | 4 | 2 |
| α-helix | 47-57 | 11 | |
| β-strand | 65-69 | 5 | 2 |
| β-strand | 76-81 | 6 | 2 |
| α-helix | 85-87 | 3 | |
| α-helix | 88-96 | 9 | |
| α-helix | 97-102 | 6 | |
| β-strand | 107-110 | 4 | 1 |
| α-helix | 117 | 1 | |
| β-strand | 118-119 | 2 | 1 |
| α-helix | 120 | 1 | |
| β-strand | 124 | 1 | 3 |
| α-helix | 127-129 | 3 | |
| α-helix | 152-167 | 16 | |
| α-helix | 173-176 | 4 | |
| α-helix | 178-180 | 3 | |
| α-helix | 181-198 | 18 | |
| β-strand | 202 | 1 | 4 |
| α-helix | 204-217 | 14 | |
| α-helix | 224-226 | 3 | |
| α-helix | 227-237 | 11 | |
| β-strand | 241-244 | 4 | 5 |
| α-helix | 248-250 | 3 | |
| α-helix | 251-259 | 9 | |
| β-strand | 262-265 | 4 | 5 |
| α-helix | 283-284 | 2 | |
| α-helix | 285-290 | 6 | |
| α-helix | 291-294 | 4 | |
| α-helix | 297-310 | 14 | |
| α-helix | 315-325 | 11 | |
| α-helix | 326-330 | 5 | |
| β-strand | 332-336 | 5 | 4 |
| β-strand | 339-342 | 4 | 4 |
| α-helix | 351-358 | 8 | |
| β-strand | 363-366 | 4 | 4 |
| α-helix | 369-372 | 4 | |
| α-helix | 382-383 | 2 | |
| β-strand | 385-389 | 5 | 4 |
| α-helix | 392-403 | 12 | |
| β-strand | 411-415 | 5 | 3 |
| β-strand | 420-424 | 5 | 3 |
| α-helix | 426-447 | 22 |
| Molecule | Chains | Type | Length | Organism | UniProt |
|---|---|---|---|---|---|
| Phenylalanine-4-hydroxylase | A, B, C, D | protein | 452 | Homo sapiens | P00439 (AlphaFold model) |
>6HYC_1 Phenylalanine-4-hydroxylase (chains A, B, C, D) MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDV NLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPW FPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQFADIAYNYRHGQPIPRVEYM EEEKKTWGTVFKTLKSLYKTHACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTCTGF RLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDRSFA QFSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKQGDSIKAYGAGLLSSFGELQYCLSE KPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVRNFAATIPRPFSVRYDPYTQR IEVLDNTQQLKILADSINSEIGILCSALQKIK
| ID | Name | Formula | Copies |
|---|---|---|---|
| H4B | 5,6,7,8-tetrahydrobiopterin | C9 H15 N5 O3 | 3 |
Structure of full-length human phenylalanine hydroxylase in complex with tetrahydrobiopterin. Flydal, M.I., Alcorlo-Pages, M., Johannessen, F.G. et al. Proc Natl Acad Sci U S A (2019) 116:11229-11234. DOI 10.1073/pnas.1902639116 · PubMed
Other PDB entries of the same protein (UniProt P00439 (AlphaFold model), which also has an AlphaFold model), best resolution first:
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