Name : Human VLDLR Protein

Product Source :
Recombinant Human VLDLR Protein is expressed from HEK293 with His tag at the C-Terminus. It contains Gly28-Ser797.[Accession | P98155-1]

Molecular Weight :
The protein has a predicted MW of 85.9 kDa. Due to glycosylation, the protein migrates to 115-125 kDa based on Tris-Bis PAGE result.

Endotoxin Level :
Less than 1EU per μg by the LAL method.

Purity :
> 95% as determined by Tris-Bis PAGE> 95% as determined by HPLC

Formulation :
Lyophilized from 0.22μm filtered solution in PBS (pH 7.4). Normally 8% trehalose is added as protectant before lyophilization.

Reconstitution :
Centrifuge the tube before opening. Reconstituting to a concentration more than 100 μg/ml is recommended. Dissolve the lyophilized protein in distilled water.

Storage and Stability :
-20 to -80°C for 12 months as supplied from date of receipt.-80°C for 3-6 months after reconstitution.2-8°C for 2-7 days after reconstitution.Recommend to aliquot the protein into smaller quantities for optimal storage. Please minimize freeze-thaw cycles.

Product Concentration :
Tris-Bis PAGE Human VLDLR on Tris-Bis PAGE under reduced condition. The purity is greater than 95%. SEC-HPLC The purity of Human VLDLR is greater than 95% as determined by SEC-HPLC. SPR Data Human VLDLR, His Tag immobilized on CM5 Chip can bind Human PCSK9, His Tag with an affinity constant of 0.72 nM as determined in SPR assay (Biacore T200).

Background :
VLDLR cerebellar hypoplasia (VLDLR-CH) is characterized by non-progressive congenital ataxia that is predominantly truncal and results in delayed ambulation, moderate-to-profound intellectual disability, dysarthria, strabismus, and seizures.VLDLR-CH is inherited in an autosomal recessive manner. Carrier testing for at-risk relatives, prenatal testing for a pregnancy at increased risk and preimplantation genetic testing are possible when the pathogenic variants in a family are known.

Synonyms :
VLDL-R; VLDLR; RP11-320E16.1; CHRMQ1; FLJ35024; VLDLRCH

References & Citations :
(1) Boycott KM, MacDonald SK, Parboosingh JS. VLDLR Cerebellar Hypoplasia. 2008 Aug 26 [updated 2020 Feb 27]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mirzaa G, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2021. PMID: 20301729.

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