Autosomal recessive hypercholesterolemia protein; LDLRAP1; ARH

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

Polyclonal

Rabbit

The antibody detects endogenous level of total LDLRAP1 polyclonal antibody.

Antigen Affinity Purified

PBS with 0.02% Sodium Azide, 50% Glycerol, pH7.3.

1.0 mg/ml (lot specific)

Store at -20 degree C

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.

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.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western Blot: 1:500 - 1:2000
Immunohistochemistry: 1:20 - 1:200

All lanes: Low density lipoprotein receptor adapter protein 1 antibody at 4ug/ml+mouse liver tissue

Immunohistochemical analysis of paraffin-embedded human liver cancer using at dilution of 1:100.

33,885 Da

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

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

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

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

Disease: Hypercholesterolemia, Autosomal Recessive

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