Human Procollagen-Lysine, 2-Oxoglutarate 5-Dioxygenase 3 (PLOD3) ELISA Kit | PLOD3 elisa kit
Human Procollagen-Lysine, 2-Oxoglutarate 5-Dioxygenase 3 (PLOD3) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Background: The LH3 cDNA clones encode a 738-amino acid polypeptide, including a signal peptide of 24 residues. The overall amino acid sequence identity between the processed human PLOD3 and PLOD1 polypeptides is 59%, and that between the processed PLOD3 and PLOD2 polypeptides is 57%, whereas the identity to the processed Caenorhabditis elegans polypeptide is only 45%. All 4 critical residues at the catalytic site, 2 histidines, 1 aspartate, and 1 arginine, are conserved in all of these polypeptides. The mRNA for PLOD3 was found to be expressed in a variety of tissues, but distinct differences appeared to exist in the expression pattern of the 3 isoenzyme mRNAs. Recombinant PLOD3 expressed in insect cells by means of a baculovirus vector was found to be more soluble than PLOD1 expressed in the same cell type.
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Jul 2008]
Uniprot Description
PLOD3: 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 PLOD3 are the cause of lysyl hydroxylase 3 deficiency (LH3 deficiency); also known as bone fragility with contractures arterial rupture and deafness. LH3 deficiency is a connective tissue disorder. The syndrome is characterized by congenital malformations severely affecting many tissues and organs and revealing features of several collagen disorders, most of them involving COL2A1 (type II collagen). The findings suggest that the failure of lysyl hydroxylation and hydroxylysyl carbohydrate addition, which affects many collagens, is the molecular basis of this syndrome.
Protein type: Oxidoreductase; Endoplasmic reticulum; Amino Acid Metabolism - lysine degradation; EC 1.14.11.4
Chromosomal Location of Human Ortholog: 7q22
Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; rough endoplasmic reticulum membrane
Molecular Function: protein binding; L-ascorbic acid binding; procollagen galactosyltransferase activity; procollagen-lysine 5-dioxygenase activity; iron ion binding; procollagen glucosyltransferase activity
Biological Process: neural tube development; extracellular matrix organization and biogenesis; protein localization; collagen fibril organization; in utero embryonic development; epidermis morphogenesis; protein modification process; endothelial cell morphogenesis; vasodilation; cellular response to hormone stimulus
Disease: Bone Fragility With Contractures, Arterial Rupture, And Deafness
Research Articles on PLOD3
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Product Notes
The Human PLOD3 plod3 (Catalog #AAA9715870) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9715870 ELISA Kit recognizes Human PLOD3. It is sometimes possible for the material contained within the vial of "Procollagen-Lysine, 2-Oxoglutarate 5-Dioxygenase 3 (PLOD3), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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