Abcam, ab223430, Alexa Fluor® 488 Anti-LEPRE1/P3H1 antibody [EPR10193(B)]

Size: 100µL
Rabbit Recombinant Monoclonal LEPRE1/P3H1 antibody - conjugated to Alexa Fluor® 488. Suitable for ICC/IF, Flow Cyt (Intra) and reacts with Human samples. Cited in 1 publication.
Key facts
Host species:Rabbit,
Clonality:Monoclonal,
Clone number:EPR10193(B),
Isotype:IgG,
Conjugation:Alexa Fluor® 488,
Excitation/Emission:Ex: 495nm, Em: 519nm,
Carrier free:No,
Reacts with:Human,
Applications:Flow Cyt (Intra), ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:The exact immunogen used to generate this antibody is proprietary information.

Product details:
Patented technology
Our RabMAb
technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to
RabMAb® patents
Alexa Fluor® is a registered trademark of Molecular Probes, Inc, a Thermo Fisher Scientific Company. The Alexa Fluor® dye included in this product is provided under an intellectual property license from Life Technologies Corporation. As this product contains the Alexa Fluor® dye, the purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). As this product contains the Alexa Fluor® dye the sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: in manufacturing; (ii) to provide a service, information, or data in return for payment (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are sold for use in research. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.4Preservative: 0.02% Sodium azideConstituents: PBS, 30% Glycerol (glycerin, glycerine), 1% BSA, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle, Stable for 12 months at -20°C, Store in the dark

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
LEPRE1 also known as P3H1 or prolyl 3-hydroxylase 1 is an enzyme involved in collagen synthesis with a molecular mass of approximately 71 kDa. It is a member of the prolyl hydroxylase family and carries out 3-hydroxylation of proline residues in collagen. The enzyme localizes largely to the endoplasmic reticulum where it contributes to the post-translational modification of collagen. LEPRE1 is ubiquitously expressed with higher concentrations observed in tissues rich in collagen such as skin bone and cartilage.
Biological function summary
LEPRE1 plays a fundamental role in the maturation of collagen which is an important structural protein in connective tissues. This enzyme functions as a part of the collagen prolyl 3-hydroxylation complex which includes cartilage-associated protein (CRTAP) and cyclophilin B (PPIB). The complex is responsible for the correct folding and stability of collagen molecules ensuring the proper formation of the extracellular matrix. This structural role is essential for maintaining tissue integrity and function.
Pathways
LEPRE1 is important in the collagen biosynthesis and modifying pathways. It interacts with other biosynthesis enzymes like lysyl oxidase and procollagen-proline dioxygenase. These proteins coordinate to execute post-translational modifications which stabilize the collagen triple helix structure. Pirh2 and Hsp47 act along with LEPRE1 in similar pathways helping in folding and secretion of collagen.
LEPRE1 mutations have been linked to connective tissue disorders most notably osteogenesis imperfecta a genetic disorder causing brittle bones. Such mutations impair collagen modification leading to defective collagen formation and bone fragility. Additionally dysregulation of LEPRE1 activity appears connected to disorder Ehlers-Danlos syndrome noted for joint hypermobility and skin elasticity issues. COL1A1 and COL1A2 two primary components of type I collagen often associate with LEPRE1 in disease contexts as mutations in these collagens lead to similar clinical manifestations.