procollagen-lysine, 2-oxoglutarate 5-dioxygenase 1

Symbol

PLOD1 (may also be known as: None)

Organism

Human

Description

Lysyl hydroxylase is a membrane-bound homodimeric protein localized to the cisternae of the endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VI have deficiencies in lysyl hydroxylase activity. [provided by RefSeq, Jul 2008]

Orthologs

SymbolSpecies
let-268 Caenorhabditis elegans
Plod Drosophila melanogaster
plod1a Danio rerio
Plod1 Mus musculus
Plod1 Rattus norvegicus

Links to external resources

Changes associated with this gene

GO Terms

GO IDGO TermGO Category
GO:0001666 response to hypoxia biological_process
GO:0006464 protein modification process biological_process
GO:0008544 epidermis development biological_process
GO:0046947 hydroxylysine biosynthetic process biological_process
GO:0055114 oxidation-reduction process biological_process
GO:0005581 collagen cellular_component
GO:0005783 endoplasmic reticulum cellular_component
GO:0016020 membrane cellular_component
GO:0030867 rough endoplasmic reticulum membrane cellular_component
GO:0005506 iron ion binding molecular_function
GO:0008475 procollagen-lysine 5-dioxygenase activity molecular_function
GO:0016702 oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen molecular_function
GO:0016706 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors molecular_function
GO:0031418 L-ascorbic acid binding molecular_function
GO:0042803 protein homodimerization activity molecular_function
GO:0046872 metal ion binding molecular_function