Anti -Low Density Lipoprotein Receptor Adapter Protein 1 (LDLRAP1, Autosomal Recessive Hypercholesterolemia Protein, ARH, ARH1, ARH2, DKFZp586D0624, FHCB1, FHCB2, MGC34705, OTTHUMP00000008526) (Azide Free)

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

Chromosome: 1; NC_000001.10 (25870071..25895377). Location: 1p36-p35

Monoclonal

IgG1,k

11C381

Mouse

Recognizes human LDLRAP1.

Purified
Purified by ammonium sulfate precipitation.

Supplied as a liquid in PBS, pH 7.2. No preservative added (Azide free).

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

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.

Antibodies; Abs to Lipoproteins

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

Suitable for use in ELISA, Immunofluorescence, Immunohistochemistry and Western Blot.
Dilution: Immunohistochemistry: 1.5ug/ml
ELISA: 3ng/ml-1ug/ml
Western Blot: 1:500-1:1000

33,885 Da[Similar Products]

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

Molecular Function: phosphatidylinositol-4,5-bisphosphate binding; protein 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; transport; amyloid precursor protein metabolic process; regulation of protein binding; receptor internalization; positive regulation of receptor-mediated endocytosis

Disease: Hypercholesterolemia, Autosomal Recessive

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