VLDLR

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.

Very Low Density Lipoprotein Receptor

Multifunctional cell surface receptor that binds VLDL and transports it into cells by endocytosis and therefore plays an important role in energy metabolism. Binds also to a wide range of other molecules including Reelin/RELN or apolipoprotein E/APOE-containing ligands as well as clusterin/CLU (PubMed:24381170, PubMed:30873003).
In the off-state of the pathway, forms homooligomers or heterooligomers with LRP8 (PubMed:30873003).
Upon binding to ligands, homooligomers are rearranged to higher order receptor clusters that transmit the extracellular RELN signal to intracellular signaling processes by binding to DAB1 (PubMed:30873003).
This interaction results in phosphorylation of DAB1 leading to the ultimate cell responses required for the correct positioning of newly generated neurons. Later, mediates a stop signal for migrating neurons, preventing them from entering the marginal zone (By similarity).

Biological Process cholesterol metabolic process Source:UniProtKB-KW
Biological Process dendrite morphogenesis Source:Ensembl
Biological Process glycoprotein transport Source:BHF-UCL1 Publication
Biological Process lipid transport Source:UniProtKB-KW
Biological Process memory Source:ProtInc1 Publication
Biological Process nervous system development Source:ProtInc1 Publication
Biological Process positive regulation of dendrite development Source:BHF-UCL
Biological Process positive regulation of protein kinase activity Source:Ensembl
Biological Process receptor-mediated endocytosis Source:BHF-UCL1 Publication
Biological Process reelin-mediated signaling pathway Source:BHF-UCL
Biological Process signal transduction Source:ProtInc1 Publication
Biological Process ventral spinal cord development Source:Ensembl
Biological Process very-low-density lipoprotein particle clearance Source:BHF-UCL1 Publication

Cell membrane
Membrane, clathrin-coated pit

Cerebellar ataxia, intellectual disability, and dysequilibrium syndrome 1 (CAMRQ1):
An autosomal recessive, congenital, non-progressive cerebellar ataxia associated with disturbed equilibrium, delayed ambulation, intellectual disability, cerebellar hypoplasia and mild cerebral gyral simplification. Additional features include short stature, strabismus, pes planus and, rarely, seizures.

Extracellular: 28-797
Helical: 798-819
Cytoplasmic: 820-873

Ubiquitinated at Lys-839 by MYLIP leading to degradation.
Glycosylated.