Low density lipoprotein receptor adapter protein 1; Autosomal recessive hypercholesterolemia protein; LDLRAP1; ARH

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

Polyclonal

Rabbit Ig

Rabbit

This ARH (LDLRAP1) antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 277-306 amino acids from the C-terminal region of human ARH (LDLRAP1).

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-LDLRAP1 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.

LDLRAP1 is a cytosolic protein which contains a phosphotyrosine binding (PTD) domain. The PTD domain has been found to interact with the cytoplasmic tail of the LDL receptor. This adapter protein is required for efficient endocytosis of the LDL receptor (LDLR) in polarized cells such as hepatocytes and lymphocytes, but not in non-polarized cells (fibroblasts). LDLRAP1 may be required for LDL binding and internalization but not for receptor clustering in coated pits. This protein may facilitate the endocytocis of LDLR and LDLR-LDL complexes from coated pits by stabilizing the interaction between the receptor and the structural components of the pits, and may also be involved in the internalization of other LDLR family members. Mutations in the LDLRAP1 gene lead to LDL receptor malfunction and cause the disorder autosomal recessive hypercholesterolaemia.

Cardiovascular; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry-Paraffin (IHC-P)

WB~~1:1000
IHC-P~~1:10~50

Western blot analysis of LDLRAP1 Antibody (C-term) in NCI-H460 cell line lysates (35ug/lane). LDLRAP1 (arrow) was detected using the purified Pab.

Formalin-fixed and paraffin-embedded human hepatocarcinoma tissue reacted with LDLRAP1 antibody (C-term) , which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.

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low density lipoprotein receptor adaptor protein 1

low density lipoprotein receptor adapter protein 1

Low density lipoprotein receptor adapter protein 1

ARH??[Similar Products]

ARH_HUMAN

The protein encoded by this gene is a cytosolic protein which contains a phosphotyrosine binding (PTD) domain. The PTD domain has been found to interact with the cytoplasmic tail of the LDL receptor. Mutations in this gene lead to LDL receptor malfunction and cause the disorder autosomal recessive hypercholesterolaemia. [provided by RefSeq, Jul 2008]

LDLRAP1: Adapter protein (clathrin-associated sorting protein (CLASP)) required for efficient endocytosis of the LDL receptor (LDLR) in polarized cells such as hepatocytes and lymphocytes, but not in non-polarized cells (fibroblasts). May be required for LDL binding and internalization but not for receptor clustering in coated pits. May facilitate the endocytocis of LDLR and LDLR-LDL complexes from coated pits by stabilizing the interaction between the receptor and the structural components of the pits. May also be involved in the internalization of other LDLR family members. Binds to phosphoinositides, which regulate clathrin bud assembly at the cell surface. Defects in LDLRAP1 are the cause of autosomal recessive hypercholesterolemia (ARH). ARH is a disorder caused by defective internalization of LDL receptors (LDLR) in the liver. ARH has the clinical features of familial hypercholesterolemia (FH) homozygotes, including severely elevated plasma LDL cholesterol, tuberous and tendon xanthomata, and premature atherosclerosis. LDL receptor (LDLR) activity measured in skin fibroblasts is normal, as the LDL binding ability.

Chromosomal Location of Human Ortholog: 1p36.11

Cellular Component: recycling endosome; internal side of plasma membrane; AP-1 adaptor complex; axon; early endosome; basal plasma membrane; neurofilament; AP-2 adaptor complex; cytosol

Molecular Function: protein binding; phosphatidylinositol-4,5-bisphosphate binding; clathrin binding; receptor signaling complex scaffold activity; phosphotyrosine binding; low-density lipoprotein receptor binding; beta-amyloid binding

Biological Process: cholesterol metabolic process; receptor-mediated endocytosis; cholesterol homeostasis; positive regulation of signal transduction; amyloid precursor protein metabolic process; transport; receptor internalization; regulation of protein binding; positive regulation of receptor-mediated endocytosis

Disease: Hypercholesterolemia, Autosomal Recessive

Maurer,M.E., J. Cell. Sci. 119 (PT 20), 4235-4246 (2006)
Keyel,P.A., Mol. Biol. Cell 17 (10), 4300-4317 (2006)

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