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Protein Page:
PLOD1 (human)

PLOD1 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. Defects in PLOD1 are the cause of Ehlers-Danlos syndrome type 6 (EDS6). EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS6 is characterized by the presence of ocular complications, particularly retinal detachment. Defects in PLOD1 are the cause of Nevo syndrome (NEVOS). This is a rare, autosomal recessive disorder characterized by increased perinatal length, kyphosis, muscular hypotonia, and joint laxity. Nevo syndrome and EDS-VI have similar clinical phenotypes. Some authors consider that both syndromes are the same clinical entity. Note: This description may include information from UniProtKB.
Protein type: Oxidoreductase; Amino Acid Metabolism - lysine degradation; EC
Cellular Component: endoplasmic reticulum membrane; rough endoplasmic reticulum membrane
Molecular Function: protein homodimerization activity; L-ascorbic acid binding; iron ion binding; procollagen-lysine 5-dioxygenase activity
Biological Process: hydroxylysine biosynthetic process; extracellular matrix organization and biogenesis; epidermis development; response to hypoxia; protein modification process
Reference #:  Q02809 (UniProtKB)
Alt. Names/Synonyms: FLJ42041; LH; LH1; LLH; lysine hydroxylase; Lysyl hydroxylase 1; PLOD; PLOD1; procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase 1; procollagen-lysine, 2-oxoglutarate 5-dioxygenase; Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1
Gene Symbols: PLOD1
Molecular weight: 83,550 Da
Basal Isoelectric point: 6.46  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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Modification Sites and Domains  

Modification Sites in Parent Protein, Orthologs, and Isoforms  

Show Multiple Sequence Alignment


SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.



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