ITN2378
ITN2378
- Catalog: ITN2378
- Gene/Protein: PLOD2
- Product Description: Immunotag™ PLOD2 Polyclonal Antibody
385.0000
Price in reward points: 385
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Antibody Specification | |
Datasheet | |
Target Protein | PLOD2 |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,ELISA |
Recommended Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
Concentration | 1 mg/ml |
Reactive Species | Human,Rat,Mouse |
Host Species | Rabbit |
Immunogen | Synthesized peptide derived from human protein, at AA range: 600-680 |
Specificity | PLOD2 Polyclonal Antibody detects endogenous levels of protein. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
Gene Name | PLOD2 |
Accession No. | O00469 Q9R0B9 Q811A3 |
Description | procollagen-lysine,2-oxoglutarate 5-dioxygenase 2(PLOD2) Homo sapiens The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough 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 VIB have deficiencies in lysyl hydroxylase activity. Mutations in the coding region of this gene are associated with Bruck syndrome. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008], |
Cell Pathway/ Category | Lysine degradation, |
Protein Expression | Aorta endothelial cell,Kidney,Liver,Lung,Placenta,P |
Subcellular Localization | endoplasmic reticulum,endoplasmic reticulum membrane,rough endoplasmic reticulum membrane,extracellular exosome, |
Protein Function | catalytic activity:Procollagen L-lysine + 2-oxoglutarate + O(2) = procollagen 5-hydroxy-L-lysine + succinate + CO(2).,cofactor:Ascorbate.,cofactor:Iron.,disease:Defects in PLOD2 are the cause of Bruck syndrome 2 (BRKS2) [MIM:609220]. Bruck syndrome [MIM:259450], also known as osteogenesis imperfecta with congenital joint contractures, is an autosomal recessive disease characterized by generalized osteopenia, joint contractures at birth, fragile bones and short stature. It can be distinguished from osteogenesis imperfecta by the absence of hearing loss and dentinogenesis imperfecta, and by the presence of clubfoot and congenital joint limitations. The molecular defect is an aberrant cross-linking of bone collagen, due to underhydroxylation of lysine residues within the telopeptides of type I collagen, whereas the lysine residues in the triple helix are normal.,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.,similarity:Contains 1 PKHD (prolyl/lysyl hydroxylase) domain.,subunit:Homodimer.,tissue specificity:Highly expressed in pancreas and muscle. Isoform 1 and isoform 2 are expressed in the majority of the examined cell types. Isoform 2 is specifically expressed in skin, lung, dura and aorta., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |