Human Prolyl 3-hydroxylase 1 (LEPRE1) ELISA kit; GROS1; MGC117314; OI8; P3H1; growth suppressor 1; leprecan; leprecan 1; prolyl 3-hydroxylase 1; leucine proline-enriched proteoglycan (leprecan) 1

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of LEPRE1 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9335828 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the leucine proline-enriched proteoglycan (leprecan) 1 (LEPRE1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing LEPRE1. The ELISA analytical biochemical technique of the MBS9335828 kit is based on LEPRE1 antibody-LEPRE1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect LEPRE1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, LEPRE1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

83,394 Da

leucine proline-enriched proteoglycan (leprecan) 1

prolyl 3-hydroxylase 1; leprecan; growth suppressor 1; leucine- and proline-enriched proteoglycan 1

Prolyl 3-hydroxylase 1

GROS1; P3H1; PSEC0109; Leprecan-1??[Similar Products]

P3H1_HUMAN

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]

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

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