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Literature summary for 1.14.11.7 extracted from

  • Vranka, J.A.; Pokidysheva, E.; Hayashi, L.; Zientek, K.; Mizuno, K.; Ishikawa, Y.; Maddox, K.; Tufa, S.; Keene, D.R.; Klein, R.; Baechinger, H.P.
    Prolyl 3-hydroxylase 1 null mice display abnormalities in fibrillar collagen-rich tissues such as tendons, skin, and bones (2010), J. Biol. Chem., 285, 17253-17262.
    View publication on PubMedView publication on EuropePMC

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
procollagen L-proline + 2-oxoglutarate + O2 Mus musculus P3H1 catalyzes the 3-hydroxylation of specific proline residues in procollagen I procollagen trans-3-hydroxy-L-proline + succinate + CO2
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Organism

Organism UniProt Comment Textmining
Mus musculus
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-
-

Source Tissue

Source Tissue Comment Organism Textmining
bone
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Mus musculus
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chondrocyte
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Mus musculus
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skin
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Mus musculus
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tendon
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Mus musculus
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
procollagen L-proline + 2-oxoglutarate + O2 P3H1 catalyzes the 3-hydroxylation of specific proline residues in procollagen I Mus musculus procollagen trans-3-hydroxy-L-proline + succinate + CO2
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?

Synonyms

Synonyms Comment Organism
P3H1
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Mus musculus
prolyl 3-hydroxylase 1
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Mus musculus

General Information

General Information Comment Organism
additional information importance of P3H1 to bone structure and development. Mutations in the gene encoding for prolyl 3-hydroxylase 1 can cause a severe, recessive form of osteogenesis imperfecta, a skeletal disorder, minimal 3-hydroxylation of key proline residues in type I collagen as a result of P3H1 deficiency. Prolyl 3-hydroxylase 1 null mice display abnormalities in fibrillar collagen-rich tissues such as tendons, skin, and bones, e.g. abnormalities in collagen fibril ultrastructure in tendons, and alterations in skin architecture, as well as in developing limbs, phenotypes, detailed overview. Collagen secretion rate is decreased in P3H1 null fibroblasts Mus musculus