Western Blot (WB)
(PLOD3 Antibody (N-term) western blot analysis in MDA-MB435 cell line lysates (35ug/lane).This demonstrates the PLOD3 antibody detected the PLOD3 protein (arrow).)
Rabbit anti-Human PLOD3 Polyclonal Antibody | anti-PLOD3 antibody
PLOD3 Antibody (N-term)
Western Blot (WB)
(PLOD3 Antibody (N-term) western blot analysis in MDA-MB435 cell line lysates (35ug/lane).This demonstrates the PLOD3 antibody detected the PLOD3 protein (arrow).)
Western Blot (WB)
(PLOD3 Antibody (N-term) western blot analysis in MDA-MB435 cell line lysates (35ug/lane).This demonstrates the PLOD3 antibody detected the PLOD3 protein (arrow).)
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.
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)
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: Amino Acid Metabolism - lysine degradation; Endoplasmic reticulum; EC 1.14.11.4; Oxidoreductase
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 PLOD3 plod3 (Catalog #AAA9205786) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 78-105. The PLOD3 Antibody (N-term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's PLOD3 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the PLOD3 plod3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PLOD3, Polyclonal Antibody" 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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