VLDL receptor

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VLDL Receptor

The VLDL receptor (VLDLR) is a protein that in humans is encoded by the VLDLR gene. It is a member of the low-density lipoprotein receptor (LDLR) family, which plays a crucial role in the metabolism of lipoproteins and the regulation of lipid homeostasis.

Structure

The VLDL receptor is structurally similar to other members of the LDL receptor family. It consists of several domains, including ligand-binding repeats, an epidermal growth factor (EGF) precursor homology domain, and a cytoplasmic domain. These structural features are important for its function in binding and internalizing lipoproteins.

Structure of LDL receptor family members, including VLDLR.

Function

The primary function of the VLDL receptor is to mediate the endocytosis of very low-density lipoproteins (VLDL) and intermediate-density lipoproteins (IDL). It is involved in the transport and metabolism of triglyceride-rich lipoproteins. The receptor is expressed in various tissues, including the heart, muscle, and adipose tissue, where it facilitates the uptake of lipoproteins for energy production and storage.

Role in Disease

The VLDL receptor has been implicated in several diseases, particularly those related to lipid metabolism. Mutations or dysregulation of the VLDLR can lead to disorders such as hyperlipidemia and atherosclerosis. Additionally, the receptor has been studied in the context of cerebral malaria, where it is thought to play a protective role.

Reelin pathway in protection from cerebral malaria.

Developmental Role

In addition to its role in lipid metabolism, the VLDL receptor is involved in the development of the central nervous system. It interacts with Reelin, a protein that regulates neuronal migration and positioning during brain development. This interaction is crucial for proper corticogenesis, the process by which the cerebral cortex is formed.

Corticogenesis in a wild-type mouse.

Related Pages

References


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Contributors: Prab R. Tumpati, MD