Background:The very-low-density-lipoprotein receptor (VLDL-R) is a lipoprotein receptor that shows considerable similarity to the lowdensity-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-densitylipoprotein (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.
仕様
サイズ:100ug
Source:Human VLDL R, His Tag (VLR-H5227) is expressed from human 293 cells (HEK293). It contains AA Gly 28 - Ser 769 (Accession # P98155-2).
Characteristics:This protein carries a polyhistidine tag at the C-terminus. The protein has a calculated MW of 84.0 kDa. The protein migrates as 90-110 kDa under reducing (R) condition (SDS-PAGE) due to different glycosylation.
Endotoxin Level:Less than 1.0 EU per ug by the LAL method.