Cytosolic PHD2,3 hydroxylates proline residues on EPAS1 (HIF2A)

Stable Identifier
Reaction [transition]
Homo sapiens
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Proline hydroxylases PHD2 (EGLN1) and PHD3 (EGLN3) located in the cytosol (Metzen et al. 2003) hydroxylate EPAS1 (HIF2A) at proline-405 and proline-531 (Hirsila et al. 2003, Percy et al. 2008, Furlow et al. 2009). A portion of PHD3 (EGLN3) is also located in the nucleus (Rantanen et al. 2008).

Literature References
PubMed ID Title Journal Year
19208626 Erythrocytosis-associated HIF-2alpha mutations demonstrate a critical role for residues C-terminal to the hydroxylacceptor proline

McMullin, MF, Furlow, PW, Percy, MJ, Lappin, TR, Bierl, C, Master, SR, Sutherland, S

J Biol Chem 2009
18184961 A gain-of-function mutation in the HIF2A gene in familial erythrocytosis

McMullin, MF, Furlow, PW, Li, X, Lucas, GS, Percy, MJ, Lappin, TR

N Engl J Med 2008
18337469 Prolyl hydroxylase PHD3 activates oxygen-dependent protein aggregation

Himanen, V, Metzen, E, Högel, H, Rantanen, K, Jaakkola, PM, Pursiheimo, J

Mol. Biol. Cell 2008
12615973 Intracellular localisation of human HIF-1 alpha hydroxylases: implications for oxygen sensing

Stengel, P, Klinger, M, Acker, H, Hellwig-Bürgel, T, Fandrey, J, Wotzlaw, C, Metzen, E, Berchner-Pfannschmidt, U, Huang, WQ, Marxsen, JH, Stolze, I, Jelkmann, W

J Cell Sci 2003
12788921 Characterization of the human prolyl 4-hydroxylases that modify the hypoxia-inducible factor

Hirsilä, M, Günzler, V, Koivunen, P, Myllyharju, J, Kivirikko, KI

J Biol Chem 2003
Catalyst Activity

peptidyl-proline 4-dioxygenase activity of PHD2,3:LIMD1,AJUBA,WTIP:VHL:EloB:EloC:CUL2:RBX1 [cytosol]

Inferred From
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