DBS; gp330; Glycoprotein 330; Megalin; Low Density Lipoprotein-Related Protein 2

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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against LRP2. It has been selected for its ability to recognize LRP2 in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-LRP2 antibody 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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western Blot: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Recombinant protein.

DABstainingonIHC-P.Samples:HumanTissue)

521,958 Da

LDL receptor related protein 2

low-density lipoprotein receptor-related protein 2

Low-density lipoprotein receptor-related protein 2

LRP-2; gp330??[Similar Products]

The protein encoded by this gene, low density lipoprotein-related protein 2 (LRP2) or megalin, is a multi-ligand endocytic receptor that is expressed in many different tissues but primarily in absorptive epithilial tissues such as the kidney. This glycoprotein has a large amino-terminal extracellular domain, a single transmembrane domain, and a short carboxy-terminal cytoplasmic tail. The extracellular ligand-binding-domains bind diverse macromolecules including albumin, apolipoproteins B and E, and lipoprotein lipase. The LRP2 protein is critical for the reuptake of numerous ligands, including lipoproteins, sterols, vitamin-binding proteins, and hormones. This protein also has a role in cell-signaling; extracellular ligands include parathyroid horomones and the morphogen sonic hedgehog while cytosolic ligands include MAP kinase scaffold proteins and JNK interacting proteins. Recycling of this membrane receptor is regulated by phosphorylation of its cytoplasmic domain. Mutations in this gene cause Donnai-Barrow syndrome (DBS) and facio-oculoacoustico-renal syndrome (FOAR).[provided by RefSeq, Aug 2009]

LRP2: Acts together with cubilin to mediate HDL endocytosis. May participate in regulation of parathyroid- hormone and para-thyroid-hormone-related protein release. Defects in LRP2 are the cause of Donnai-Barrow syndrome (DBS); also known as faciooculoacousticorenal syndrome (FOAR syndrome). DBS is a rare autosomal recessive disorder characterized by major malformations including agenesis of the corpus callosum, congenital diaphragmatic hernia, facial dysmorphology, ocular anomalies, sensorineural hearing loss and developmental delay. The FOAR syndrome was first described as comprising facial anomalies, ocular anomalies, sensorineural hearing loss, and proteinuria. DBS and FOAR were first described as distinct disorders but the classic distinguishing features between the 2 disorders were presence of proteinuria and absence of diaphragmatic hernia and corpus callosum anomalies in FOAR. Early reports noted that the 2 disorders shared many phenotypic features and may be identical. Although there is variability in the expression of some features (e.g. agenesis of the corpus callosum and proteinuria), DBS and FOAR are now considered to represent the same entity. Belongs to the LDLR family.

Protein type: Membrane protein, integral; Motility/polarity/chemotaxis; Receptor, misc.

Chromosomal Location of Human Ortholog: 2q31.1

Cellular Component: apical plasma membrane; axon; brush border membrane; clathrin-coated pit; clathrin-coated vesicle membrane; dendrite; endocytic vesicle; endoplasmic reticulum; endosome; external side of plasma membrane; Golgi apparatus; integral component of membrane; lysosomal membrane; lysosome; plasma membrane; receptor complex

Molecular Function: calcium ion binding; chaperone binding; drug binding; lipoprotein transporter activity; low-density lipoprotein receptor activity; protein binding; SH3 domain binding; steroid hormone receptor binding

Biological Process: cell proliferation; endocytosis; forebrain development; lipid metabolic process; lipoprotein transport; male gonad development; metal ion transport; negative regulation of BMP signaling pathway; neural tube closure; positive regulation of neurogenesis; receptor-mediated endocytosis; retinoid metabolic process; sensory perception of sound; vagina development; vitamin D metabolic process

Disease: Donnai-barrow Syndrome

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