antibody

Mouse Anti-Human Megalin

MBS690201

0.1 mg

365 USD

monoclonal

mouse

nonconjugated

IgG1; CD7D5

immunohistochemistry, immunocytochemistry

Antibody

Mouse Anti-Human Megalin

Megalin

Anti-human Megalin/gp330 (#CD7D5); Low-density lipoprotein receptor-related protein 2; Glycoprotein 330; gp330

low-density lipoprotein receptor-related protein 2; Low-density lipoprotein receptor-related protein 2; low-density lipoprotein receptor-related protein 2; LRP-2; megalin; glycoprotein 330; calcium sensor protein; Heymann nephritis antigen homolog; low density lipoprotein receptor-related protein 2; Glycoprotein 330; gp330; Megalin

LRP2; LRP2; DBS; GP330; LRP-2; gp330

LRP2_HUMAN

Monoclonal

IgG1

IgG1; CD7D5

Mouse

Human

4655

The antibody cross-reacts with rat and mouse Magalin.

Protein G purified

Lyophilized

The lyophilized antibody is stable at room temperature for up to 1 month. The reconstituted antibody is stable for at least two weeks at 2-8 degree C. Frozen aliquots are stable for at least 6 months when stored at -20 degree C. AVOID REPEATED FREEZE AND THAW CYCLES!

Immunohistochemistry (IHC), Immunofluorescence (IF)

IF/IHC: use at 2-5ug/ml

Immunogen: purified human Megalin. Preparation: Monoclonal antibodies were produced with the help of BALB/c mice using a membrane protein fraction of isolated human glomeruli as the immunizing antigen. Reconstitution: Centrifuge vial prior to opening. Reconstitute in sterile water to a concentration of 0.1-1.0 mg/ml.

Buffer: PBS

Megalin/gp330, an approx. 600 kDa transmembrane glycoprotein is a large cell surface receptor that mediates the binding and internalization of a number of structurally and functionally distinct ligands from the lipoprotein and protease:protease inhibitor families.

1. Farquhar et al., J Am Soc Nephrol. 6:35, 1995 2. Saito et al., PNAS 93:8601, 1996 3. Hiesberger et al., JBC 271:28792, 1996 4. Orlando et al., PNAS 94:2368, 1997 5. Kerjaschki et al., J Clin Invest. 100:2303, 1997 6. Ziak et al., J Am Soc Nephrol. 10:203, 1999

126012573

NP_004516.2

NM_004525.2

P98164

521,958 Da

Disease Pathway (530764); Diseases Associated With Visual Transduction Pathway (771581); Hedgehog Signaling Pathway (83063); Hedgehog Signaling Pathway (474); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Metabolism Of Steroid Hormones And Vitamin D Pathway (106150); Retinoid Metabolism And Transport Pathway (187208); Signal Transduction Pathway (477114); Signaling Events Mediated By The Hedgehog Family Pathway (137950)

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]

Function: Acts together with cubilin to mediate HDL endocytosis . By similarity. May participate in regulation of parathyroid-hormone and para-thyroid-hormone-related protein release. Subunit structure: Binds plasminogen, extracellular matrix components, plasminogen activator-plasminogen activator inhibitor type I complex, apolipoprotein E-enriched beta-VLDL, lipoprotein lipase, lactoferrin, CLU/clusterin and calcium. Forms a multimeric complex together with a receptor-associated protein (RAP). Binds to ankyrin-repeat family A protein 2 (ANKRA2). Interacts with LRP2BP. Interacts (via NPXY motif) with DAB2; the interaction is not affected by tyrosine phosphorylation of the NPXY motif. Ref.5 Ref.6 Ref.7 Ref.8. Subcellular location: Membrane; Single-pass type I membrane protein. Membrane coated pit. Tissue specificity: Absorptive epithelia, including renal proximal tubules. Involvement in disease: Donnai-Barrow syndrome (DBS) [MIM:222448]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10. Sequence similarities: Belongs to the LDLR family.Contains 17 EGF-like domains.Contains 36 LDL-receptor class A domains.Contains 37 LDL-receptor class B repeats.

0.1 mg

365 USD