VLDL Receptor/VLDLR Active Protein | VLDLR active protein

VLDL Receptor/VLDLR Protein, Human, Recombinant (His Tag)

Cat. Num. AAA8120674

• Gene Names: VLDLR; CARMQ1; CHRMQ1; VLDLRCH
• Purity: >80% as determined by SDS-PAGE
• Synonyms: VLDL Receptor/VLDLR; VLDL Receptor/VLDLR Protein; Human; Recombinant (His Tag); Human VLDLR/VLDL Receptor Protein (His Tag); CAMRQ1 Protein; CARMQ1 Protein; CHRMQ1 Protein; VLDLRCH Protein; very low density lipoprotein receptor; VLDLR active protein

• Host: HEK293 Cells
• Purity/Purification: >80% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Ser797

• Species: Human
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Gly 28
• Tag: C-His
• Protein Construction: A DNA sequence encoding the human VLDLR isoform alpha (NP_003374.3) extracellular domain (Met 1-Ser 797) was fused with a polyhistidine tag at the C-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Activity: 1.Measured by its binding ability in a functional ELISA.2.Immobilized human VLDLR-His at 10ug/mL (100uL/well) can bind biotinylated human LRPAP1-His. The EC50 of biotinylated human LRPAP1-His is 0.05-0.2ug/mL.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Testing Data

(Measured by its binding ability in a functional ELISA. Immobilized human VLDLR-His at 10ug/mL (100uL/well) can bind biotinylated human LRPAP1-His, the EC50 of biotinylated human LRPAP1-His is 0.05-0.2 ug/mL.)

Related Product Information for VLDLR active protein

Background: The very low density lipoprotein receptor, known as VLDLR, is a single-pass type 1 integral membrance protein and a member of the LDL receptor family. This receptor family includes LDL receptor, LRP, megalin, VLDLR and ApoER2, and is characterized by a cluster of cysteine-rich class A repeats, epidermal growth factor (EGF)-like repeats, YWTD repeats and an O-linked sugar sdomain. VLDLR contains 3 EGF-like domains, 8 LDL-receptor class A domains, as well as 6 LDL-receptor class B repeats, and is abundant in heart, skeletal muscle, also ovary and kidney, but not in liver. 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. VLDLR mediates the phosphorylation of mDab1 (mammalian disabled protein) via binding to Reelin, and induces the modulation of Tau phosphorylation. This pathway regulates the migration of neurons along with the radial glial fiber network during brain development. Defects of VLDLR may be the cause of VLDLR-associated cerebellar hypoplasia (VLDLRCH), a syndrome characterized by moderate-to-profound mental retardation, delayed ambulation, and predominantly truncal ataxia.

References

Trommsdorff, M. et al., 1999. Cell. 97: 689-701. Mikhailenko, I. et al., 1999. J. Cell Sci. 112: 3269-3281. Sato, A. et al., 1999. Biochem. J. 341: 377-383. Hiesberger, T. et al., 1999. Neuron 24: 481-489. Tiebel, O. et al., 1999. Atherosclerosis 145: 239-251. Boycott, K.M. et al., 2005, Am. J. Hum. Genet. 77 (3): 477-483. Moheb, L.A. et al., 2008, Eur. J. Hum. Genet. 16 (2): 270-273.

NCBI and Uniprot Product Information

• NCBI GI #: 65301167
• NCBI GeneID: 7436
• NCBI Accession #: NP_003374.3
• NCBI GenBank Nucleotide #: NM_003383.3
• UniProt Accession #: P98155; Q5VVF6; B2RMZ7; D3DRH6
• Molecular Weight: 96,098 Da
• NCBI Official Full Name: very low-density lipoprotein receptor isoform a
• NCBI Official Synonym Full Names: very low density lipoprotein receptor
• NCBI Official Symbol: VLDLR
• NCBI Official Synonym Symbols: CARMQ1; CHRMQ1; VLDLRCH
• NCBI Protein Information: very low-density lipoprotein receptor; VLDL-R; VLDL 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: Membrane protein, integral; Receptor, misc.

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: nervous system development; receptor-mediated endocytosis; 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