Human leucine proline-enriched proteoglycan (leprecan) 1 ELISA Kit | LEPRE1 elisa kit
Human Prolyl 3-hydroxylase 1, LEPRE1 ELISA Kit
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: Secreted, signal peptide; Oxidoreductase; Secreted; Cell development/differentiation; EC 1.14.11.7
Chromosomal Location of Human Ortholog: 1p34.1
Cellular Component: proteinaceous extracellular matrix; membrane; endoplasmic reticulum lumen; endoplasmic reticulum; nucleus
Molecular Function: collagen binding; procollagen-proline 3-dioxygenase activity; L-ascorbic acid binding; iron ion binding; protein complex binding
Biological Process: negative regulation of cell proliferation; extracellular matrix organization and biogenesis; collagen fibril organization; regulation of protein secretion; protein stabilization; collagen metabolic process; protein folding; peptidyl-proline hydroxylation; protein amino acid hydroxylation; regulation of ossification; cell growth
Disease: Osteogenesis Imperfecta, Type Viii
Research Articles on LEPRE1
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Product Notes
The Human LEPRE1 lepre1 (Catalog #AAA9335828) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9335828 ELISA Kit recognizes Human LEPRE1. It is sometimes possible for the material contained within the vial of "leucine proline-enriched proteoglycan (leprecan) 1, 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
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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