VLDL receptor: Difference between revisions

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'''Very Low-Density Lipoprotein Receptor''' ('''VLDL receptor''') is a protein that in humans is encoded by the ''VLDLR'' gene. The VLDL receptor is a key component in the regulation of lipoprotein metabolism and plays a significant role in the process of clearing triglyceride-rich lipoproteins from the bloodstream. It is closely related to the LDL receptor and is involved in the pathophysiology of several metabolic disorders, including cardiovascular diseases and obesity.
== VLDL Receptor ==


==Function==
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.
The VLDL receptor is a cell surface protein that binds to and internalizes triglyceride-rich lipoproteins such as VLDL (very low-density lipoproteins) and chylomicron remnants. This process is essential for the clearance of these lipoproteins from the bloodstream, facilitating their delivery to cells for energy production or storage. The receptor is predominantly expressed in tissues with high fatty acid metabolism, including heart, muscle, and adipose tissue. It also plays a role in the brain, where it is involved in the transport of lipoproteins across the blood-brain barrier and may influence neuronal development and function.


==Structure==
== Structure ==
The VLDL receptor is a type I membrane protein that belongs to the LDL receptor family. It is characterized by a modular structure, including an extracellular domain with ligand-binding repeats, EGF-precursor homology domains, an O-linked sugar domain, a transmembrane domain, and a cytoplasmic domain. The ligand-binding repeats are crucial for the interaction with lipoproteins, while the cytoplasmic domain is involved in the internalization and recycling of the receptor.


==Clinical Significance==
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.
Alterations in the function or expression of the VLDL receptor can contribute to the development of metabolic diseases. For example, reduced receptor activity can lead to the accumulation of VLDL and chylomicron remnants in the bloodstream, increasing the risk of atherosclerosis and cardiovascular disease. Furthermore, the VLDL receptor has been implicated in the pathogenesis of obesity and type 2 diabetes, as it influences the distribution and storage of fatty acids in tissues.


==Genetics==
[[File:Structure_of_LDL_receptor_family_members.png|thumb|right|Structure of LDL receptor family members, including VLDLR.]]
The ''VLDLR'' gene is located on chromosome 9p24 and consists of multiple exons that encode the different domains of the receptor. Genetic variations in ''VLDLR'' have been associated with differences in lipid levels among individuals and susceptibility to metabolic diseases. Additionally, mutations in the ''VLDLR'' gene can lead to rare genetic disorders, such as dysequilibrium syndrome, characterized by non-progressive cerebellar ataxia and cognitive impairment.


==Research Directions==
== Function ==
Research on the VLDL receptor continues to explore its role in lipid metabolism and its potential as a therapeutic target for metabolic diseases. Studies are investigating the effects of modulating VLDL receptor activity on lipid levels, insulin sensitivity, and the progression of atherosclerosis. Additionally, the receptor's role in the brain opens avenues for research into its involvement in neurodegenerative diseases and cognitive 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.
[[File:Reelin_pathway_in_protection_from_Cerebral_Malaria_PMID_18062806.jpg|thumb|right|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.
[[File:Corticogenesis_in_a_wild-type_mouse_with_captions_in_english_copy.png|thumb|right|Corticogenesis in a wild-type mouse.]]
== Related Pages ==
* [[Low-density lipoprotein receptor]]
* [[Reelin]]
* [[Lipoprotein]]
* [[Cerebral malaria]]
== References ==
{{Reflist}}
[[Category:Receptors]]
[[Category:Lipoproteins]]
[[Category:Lipoproteins]]
[[Category:Membrane biology]]
[[Category:Human proteins]]
[[Category:Genetics]]
<gallery>
[[Category:Cardiovascular diseases]]
File:Structure_of_LDL_receptor_family_members.png|Structure of LDL receptor family members
[[Category:Metabolic disorders]]
File:Reelin_pathway_in_protection_from_Cerebral_Malaria_PMID_18062806.jpg|Reelin pathway in protection from Cerebral Malaria
 
File:Corticogenesis_in_a_wild-type_mouse_with_captions_in_english_copy.png|Corticogenesis in a wild-type mouse
{{medicine-stub}}
</gallery>

Latest revision as of 04:17, 18 February 2025

VLDL Receptor[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

References[edit]

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