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Literature summary for 1.14.11.7 extracted from

  • Hudson, D.M.; Joeng, K.S.; Werther, R.; Rajagopal, A.; Weis, M.; Lee, B.H.; Eyre, D.R.
    Post-translationally abnormal collagens of prolyl 3-hydroxylase-2 null mice offer a pathobiological mechanism for the high myopia linked to human LEPREL1 mutations (2015), J. Biol. Chem., 290, 8613-8622 .
    View publication on PubMedView publication on EuropePMC
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Protein Variants

Protein Variants Comment Organism
additional information generation of mutant mice with mutations in LEPREL1, the gene encoding prolyl 3-hydroxylase-2 (P3H2), causing severe nonsyndromic myopia, phenotype, overview Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Mus musculus type IV collagen contains more prolyl 3-hydroxylation sites than any other collagen types ?
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[procollagen]-L-proline + 2-oxoglutarate + O2 Mus musculus prolyl 3-hydroxylation in lens capsule, prolyl 3-hydroxylation at Pro602 from alpha1(IV) and Pro197 from alpha2(IV). Pro707 site in alpha1(I) is a tissue-specific substrate unique to P3h2 [procollagen]-trans-3-hydroxy-L-proline + succinate + CO2
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Organism

Organism UniProt Comment Textmining
Mus musculus Q8CG71
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Source Tissue

Source Tissue Comment Organism Textmining
eye lens capsule Mus musculus
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information type IV collagen contains more prolyl 3-hydroxylation sites than any other collagen types Mus musculus ?
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 ?
[procollagen]-L-proline + 2-oxoglutarate + O2 prolyl 3-hydroxylation in lens capsule, prolyl 3-hydroxylation at Pro602 from alpha1(IV) and Pro197 from alpha2(IV). Pro707 site in alpha1(I) is a tissue-specific substrate unique to P3h2 Mus musculus [procollagen]-trans-3-hydroxy-L-proline + succinate + CO2
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 ?
[procollagen]-L-proline + 2-oxoglutarate + O2 collagen from bovine tissue. 3-hydroxyproline occupancy in collagens from bovine and mouse tissues, overview Mus musculus [procollagen]-trans-3-hydroxy-L-proline + succinate + CO2
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 ?

Synonyms

Synonyms Comment Organism
Leprel1
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 Mus musculus
P3H2
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 Mus musculus
prolyl 3-hydroxylase-2
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 Mus musculus

General Information

General Information Comment Organism
malfunction mutations in LEPREL1, the gene encoding prolyl 3-hydroxylase-2 (P3H2), cause severe nonsyndromic myopia. Collagens I and IV from P3h2-null mouse eye tissues are significantly reduced in 3-hydroxylation compared with wild-type littermates. Loss of P3h2 causes altered collagen prolyl 3-hydroxylation from multiple tissues, e.g. leading to structural abnormalities in multiple eye tissues, but particularly sclera, causing progressive myopia. Phenotype, overview Mus musculus
physiological function role for prolyl 3-hydroxylase-2, P3H2, in collagen IV prolyl 3-hydroxylation. Collagen IV is found in many tissues in the eye, including lens capsule, epidermal and endodermal membranes of the cornea, and the inner limiting membrane and Bruch’s membrane of the retina Mus musculus