Lysyl hydroxylase
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| procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase 1
| |
| Identifiers | |
| Symbol | PLOD1 |
| Alt. Symbols | LLH, PLOD |
| Entrez | 5351 |
| HUGO | 9081 |
| OMIM | 153454 |
| RefSeq | NM_000302 |
| UniProt | Q02809 |
| Other data | |
| EC number | 1.14.11.4 |
| Locus | Chr. 1 p36.3-36.2 |
| procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2
| |
| Identifiers | |
| Symbol | PLOD2 |
| Entrez | 5352 |
| HUGO | 9082 |
| OMIM | 601865 |
| RefSeq | NM_000935 |
| UniProt | O00469 |
| Other data | |
| Locus | Chr. 3 q24 |
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Overview
Lysyl hydroxylase (or procollagen-lysine 5-dioxygenase) is an oxygenase enzyme which catalyzes the hydroxylation of lysine to hydroxylysine. This reaction is involved in the formation and stabilization of collagen.
It requires vitamin C as a cofactor.
Pathology
A deficiency is associated with Ehlers-Danlos syndrome.
External links
Oxidoreductases: dioxygenases, including steroid hydroxylases (EC 1.14) | |
|---|---|
| 1.14.11 - 2-oxoglutarate | Prolyl hydroxylase - Lysyl hydroxylase |
| 1.14.13 - NADH or NADPH | Flavin-containing monooxygenase - Nitric oxide synthase - Cholesterol 7 alpha-hydroxylase - Methane monooxygenase - 3A4 -51A1 |
| 1.14.14 - reduced flavin or flavoprotein | 19A1 - 2D6 - 2E1 |
| 1.14.15 - reduced iron-sulfur protein | 11B1 - 11B2 - 11A1 |
| 1.14.16 - reduced pteridine | Phenylalanine hydroxylase - Tyrosine hydroxylase - Tryptophan hydroxylase |
| 1.14.17 - reduced ascorbate | Dopamine beta hydroxylase |
| 1.14.18-19 - other | Tyrosinase - Stearoyl-CoA desaturase-1 |
| 1.14.99 - miscellaneous | Cyclooxygenase - Heme oxygenase (HMOX1) - Squalene monooxygenase - 17A1 - 21A2 |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
