|Other Names||Procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3, Lysyl hydroxylase 3, LH3, PLOD3|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.|
|Cellular Location||Rough endoplasmic reticulum membrane; Peripheral membrane protein; Lumenal side|
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Provided below are standard protocols that you may find useful for product applications.
The protein encoded by this gene is a membrane-boundhomodimeric enzyme that is localized to the cisternae of the roughendoplasmic reticulum. The enzyme (cofactors iron and ascorbate)catalyzes the hydroxylation of lysyl residues in collagen-likepeptides. The resultant hydroxylysyl groups are attachment sitesfor carbohydrates in collagen and thus are critical for thestability of intermolecular crosslinks. Some patients withEhlers-Danlos syndrome type VIB have deficiencies in lysylhydroxylase activity.
Wang, C., et al. J. Cell. Mol. Med. 13(3):508-521(2009)Salo, A.M., et al. Am. J. Hum. Genet. 83(4):495-503(2008)Salo, A.M., et al. J. Cell. Physiol. 207(3):644-653(2006)Wang, C., et al. Matrix Biol. 21(7):559-566(2002)Rautavuoma, K., et al. J. Biol. Chem. 277(25):23084-23091(2002)
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