LDLRAP1 Human; Low Density Lipoprotein Receptor Adaptor Protein 1 Human Recombinant; Low density lipoprotein receptor adaptor protein 1; Autosomal recessive hypercholesterolemia protein; ARH1; FHCB1; FHCB2; ARH2; LDL receptor adaptor protein; MGC34705; DKFZp586D0624

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

E Coli

Greater than 90% as determined by SDS-PAGE.

The LDLRAP1 solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 200mM NaCl, 2mM DTT and 10% glycerol.
Sterile Filtered colorless solution.

Small volumes of LDLRAP1 recombinant protein 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.

Description: LDLRAP1 Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 328 amino acids (1-308) and having a molecular mass of 36.1kDa.LDLRAP1 is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Introduction: LDLRAP1 is a cytosolic protein that holds a phosphotyrosine binding (PTD) domain and performing as a cytosolic adaptor it couples LDLR to endocytic machinery. Mutations in LDLRAP1 are the reason for autosomal recessive hypercholesterolemia (ARH) which is a condition triggered by defective internalization of LDL receptors (LDLR) in the liver.

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins

33,885 Da

low density lipoprotein receptor adaptor protein 1

low density lipoprotein receptor adapter protein 1; LDL receptor adaptor protein; autosomal recessive hypercholesterolemia protein

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; neurofilament; basal plasma membrane; AP-2 adaptor complex; cytosol

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

Biological Process: receptor-mediated endocytosis; cholesterol metabolic process; 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

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