VLDLR active protein

Human CellExp VLDLR, human recombinant

Cat. Num. AAA844463

• Gene Names: VLDLR; CAMRQ1; CARMQ1; CHRMQ1; VLDLRCH
• Applications: SDS-Page
• Purity: >=97%
• Synonyms: VLDLR; Human CellExp VLDLR; human recombinant; CARMQ1; CHRMQ1; FLJ35024; VLDLRCH; VLDL-R; very-low-density-lipoprotein receptor; VLDLR active protein

• Host: HEK293 cells
• Purity/Purification: >=97%
• Form/Format: Lyophilized from 0.22 mum filtered solution in PBS, pH 7.4. Normally Mannitol or Trehalose is added as protectants before lyophilization.; Appearance: Lyophilized
• Sequence Length: 845

• Applicable Applications for VLDLR active protein: SDS-PAGE
• Solubility/Reconstitution Instructions: Centrifuge the vial prior to opening. Reconstitute in sterile PBS, pH 7.4 to a concentration of 50 ug/ml. Do not vortex. This solution can be stored at 2-8 degree C for up to 1 month. For extended storage, it is recommended to store at -20 degree C.
• Endotoxin Level: <1 EU/mug by LAL method
• Biological Activity: Measured by its binding ability in a functional ELISA. When Recombinant Human Apolipoprotein E3 is immobilized at 1 mug/ml (100 mul/well), the concentration of Recombinant Human VLDLR that produces 50% of the optimal binding response is found to be approximately 0.03 - 0.15 mug/ml.
• Results: Measured by its binding ability in a functional ELISA. When Recombinant Human Apolipoprotein E3 is immobilized at 1 mug/ml (100 mul/well), the concentration of Recombinant Human VLDLR that produces 50% of the optimal binding response is found to be approximately 0.03 - 0.15 mug/ml.
• Handling: Centrifuge the vial prior to opening.
• Preparation and Storage: At -20 degree C; Shelf Life: 12 months

Testing Data

Related Product Information for VLDLR active protein

Background: The very-low-density-lipoprotein receptor (VLDL-R) is a lipoprotein receptor that shows considerable similarity to the low density-lipoprotein receptor. VLDL R is a 130 kDa type I transmembrane protein in the LDL receptor family that plays a significant role in lipid metabolism and in nervous system development and function.This receptor has been suggested to be important for the metabolism of apoprotein-E-containing triacylglycerol-rich lipoproteins, such as very-low-density lipoprotein (VLDL), beta-migrating VLDL and intermediate-density lipoprotein. It is also one of the receptors of reelin, an extracellular matrix protein which regulates the processes of neuronal migration and synaptic plasticity. In humans, the VLDL-R is encoded by the VLDLR gene. A rare neurological disorder first described in the 1970s under the name "disequilibrium syndrome" is now considered to be caused by the disruption of VLDLR gene. The disorder was renamed VLDLR-associated cerebellar hypoplasia (VLDLRCH) after a 2005 study. It is associated with parental consanguinity and found in secluded communities such as the Hutterites. VLDLRCH is one of the two known genetic disorders caused by a disruption of reelin signaling pathway, along with Norman-Roberts syndrome.

Product Categories/Family for VLDLR active protein

Proteins and Enzymes; Human cell expressed recombinant proteinsProteins and Enzymes; Proteins and Enzymes (A-Z) Proteins and Enzymes; Other Proteins & EnzymesDiabetes; Obesity & Metabolic Syndrome; Lipids Metabolism; Proteins/Enzymes; Other (NEW)

NCBI and Uniprot Product Information

• NCBI GI #: 65301164
• NCBI GeneID: 7436
• NCBI Accession #: NP_001018066.1
• NCBI GenBank Nucleotide #: NM_001018056.1
• UniProt Accession #: P98155; Q5VVF6; B2RMZ7; D3DRH6
• Molecular Weight: This protein comprises 781 amino acids with polyhistidine tag at C-terminus and has a calculated MW of 86 kDa. The predicted N-terminus is Gly 28. DTT-reduced protein migrates as 150 & 180 kDa polypeptide in SDS-PAGE due to different glycosylation.
• NCBI Official Full Name: very low-density lipoprotein receptor isoform b
• NCBI Official Synonym Full Names: very low density lipoprotein receptor
• NCBI Official Symbol: VLDLR
• NCBI Official Synonym Symbols: CAMRQ1; CARMQ1; CHRMQ1; VLDLRCH
• NCBI Protein Information: very low-density lipoprotein receptor
• UniProt Protein Name: Very low-density lipoprotein receptor
• Protein Family: Very low-density lipoprotein receptor
• UniProt Gene Name: VLDLR
• UniProt Synonym Gene Names: VLDL receptor; VLDL-R
• UniProt Entry Name: VLDLR_HUMAN

NCBI Description

The low density lipoprotein receptor (LDLR) gene family consists of cell surface proteins involved in receptor-mediated endocytosis of specific ligands. This gene encodes a lipoprotein receptor that is a member of the LDLR family and plays important roles in VLDL-triglyceride metabolism and the reelin signaling pathway. Mutations in this gene cause VLDLR-associated cerebellar hypoplasia. Alternative splicing generates multiple transcript variants encoding distinct isoforms for this gene. [provided by RefSeq, Aug 2009]

Uniprot Description

VLDLR: Binds VLDL and transports it into cells by endocytosis. In order to be internalized, the receptor-ligand complexes must first cluster into clathrin-coated pits. Binding to Reelin induces tyrosine phosphorylation of Dab1 and modulation of Tau phosphorylation. Defects in VLDLR are the cause of cerebellar ataxia mental retardation and dysequilibrium syndrome type 1 (CMARQ1); also known as dysequilibrium syndrome (DES) or non- progressive cerebellar disorder with mental retardation. CMARQ1 is a congenital, non-progressive cerebellar ataxia associated with disturbed equilibrium, delayed ambulation, mental retardation and cerebellar hypoplasia. Additional features include short stature, strabismus, pes planus and, rarely, seizures. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Receptor, misc.; Membrane protein, integral

Chromosomal Location of Human Ortholog: 9p24

Cellular Component: membrane; plasma membrane; integral to membrane; coated pit; receptor complex

Molecular Function: very-low-density lipoprotein receptor activity; low-density lipoprotein receptor activity; very-low-density lipoprotein binding; protein binding; apolipoprotein binding; calcium ion binding; glycoprotein binding; calcium-dependent protein binding

Biological Process: receptor-mediated endocytosis; nervous system development; cholesterol metabolic process; positive regulation of protein kinase activity; ventral spinal cord development; negative regulation of transcription from RNA polymerase II promoter; signal transduction; lipid transport; memory

Disease: Cerebellar Ataxia, Mental Retardation, And Dysequilibrium Syndrome 1