Rabbit anti-Human, Mouse LEPRE1 Polyclonal Antibody | anti-LEPRE1 antibody
LEPRE1 Polyclonal Antibody
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using LEPRE1 antibody at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 30s.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an enzyme that is a member of the collagen prolyl hydroxylase family. These enzymes are localized to the endoplasmic reticulum and their activity is required for proper collagen synthesis and assembly. Mutations in this gene are associated with osteogenesis imperfecta type VIII. Three alternatively spliced transcript variants encoding different isoforms have been described. Other variants may exist, but their biological validity has not been determined. [provided by RefSeq, Aug 2011]
Uniprot Description
LEPRE1: Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro- Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts. Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8). A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest. Belongs to the leprecan family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Cell development/differentiation; EC 1.14.11.7; Oxidoreductase; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 1p34.2
Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; membrane
Molecular Function: collagen binding; procollagen-proline 3-dioxygenase activity; protein complex binding
Biological Process: collagen metabolic process; protein amino acid hydroxylation; protein folding; protein stabilization; regulation of protein secretion
Disease: Osteogenesis Imperfecta, Type Viii
Research Articles on LEPRE1
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Product Notes
The LEPRE1 p3h1 (Catalog #AAA9130833) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The LEPRE1 Polyclonal Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's LEPRE1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:1000 - 1:2000. Researchers should empirically determine the suitability of the LEPRE1 p3h1 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 "LEPRE1, 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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