Rabbit anti-Human LLH Polyclonal Antibody | anti-PLOD1 antibody
Anti-LLH Antibody
NCBI and Uniprot Product Information
NCBI 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. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2015]
Uniprot Description
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.
Protein type: Oxidoreductase; Amino Acid Metabolism - lysine degradation; EC 1.14.11.4
Chromosomal Location of Human Ortholog: 1p36.22
Cellular Component: endoplasmic reticulum membrane; rough endoplasmic reticulum membrane
Molecular Function: ferrous iron binding; L-ascorbic acid binding; peptide binding; procollagen-lysine 5-dioxygenase activity; protein homodimerization activity
Biological Process: cellular response to hormone stimulus; epidermis development; hydroxylysine biosynthetic process; peptidyl-lysine hydroxylation; protein modification process; response to hypoxia
Disease: Ehlers-danlos Syndrome, Type Vi
Research Articles on PLOD1
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Product Notes
The PLOD1 plod1 (Catalog #AAA8245116) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-LLH Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's LLH can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1/500 - 1/2000. Researchers should empirically determine the suitability of the PLOD1 plod1 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 "LLH, 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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