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ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions
The α/β-Hydrolase domain-containing protein 5 (ABHD5; also known as comparative gene identification-58, or CGI-58) is the causative gene of the Chanarin-Dorfman syndrome (CDS), a disorder mainly characterized by systemic triacylglycerol accumulation and a severe defect in skin barrier function. The...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9694394/ https://www.ncbi.nlm.nih.gov/pubmed/36355098 http://dx.doi.org/10.3390/metabo12111015 |
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author | Schratter, Margarita Lass, Achim Radner, Franz P. W. |
author_facet | Schratter, Margarita Lass, Achim Radner, Franz P. W. |
author_sort | Schratter, Margarita |
collection | PubMed |
description | The α/β-Hydrolase domain-containing protein 5 (ABHD5; also known as comparative gene identification-58, or CGI-58) is the causative gene of the Chanarin-Dorfman syndrome (CDS), a disorder mainly characterized by systemic triacylglycerol accumulation and a severe defect in skin barrier function. The clinical phenotype of CDS patients and the characterization of global and tissue-specific ABHD5-deficient mouse strains have demonstrated that ABHD5 is a crucial regulator of lipid and energy homeostasis in various tissues. Although ABHD5 lacks intrinsic hydrolase activity, it functions as a co-activating enzyme of the patatin-like phospholipase domain-containing (PNPLA) protein family that is involved in triacylglycerol and glycerophospholipid, as well as sphingolipid and retinyl ester metabolism. Moreover, ABHD5 interacts with perilipins (PLINs) and fatty acid-binding proteins (FABPs), which are important regulators of lipid homeostasis in adipose and non-adipose tissues. This review focuses on the multifaceted role of ABHD5 in modulating the function of key enzymes in lipid metabolism. |
format | Online Article Text |
id | pubmed-9694394 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-96943942022-11-26 ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions Schratter, Margarita Lass, Achim Radner, Franz P. W. Metabolites Review The α/β-Hydrolase domain-containing protein 5 (ABHD5; also known as comparative gene identification-58, or CGI-58) is the causative gene of the Chanarin-Dorfman syndrome (CDS), a disorder mainly characterized by systemic triacylglycerol accumulation and a severe defect in skin barrier function. The clinical phenotype of CDS patients and the characterization of global and tissue-specific ABHD5-deficient mouse strains have demonstrated that ABHD5 is a crucial regulator of lipid and energy homeostasis in various tissues. Although ABHD5 lacks intrinsic hydrolase activity, it functions as a co-activating enzyme of the patatin-like phospholipase domain-containing (PNPLA) protein family that is involved in triacylglycerol and glycerophospholipid, as well as sphingolipid and retinyl ester metabolism. Moreover, ABHD5 interacts with perilipins (PLINs) and fatty acid-binding proteins (FABPs), which are important regulators of lipid homeostasis in adipose and non-adipose tissues. This review focuses on the multifaceted role of ABHD5 in modulating the function of key enzymes in lipid metabolism. MDPI 2022-10-24 /pmc/articles/PMC9694394/ /pubmed/36355098 http://dx.doi.org/10.3390/metabo12111015 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Schratter, Margarita Lass, Achim Radner, Franz P. W. ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions |
title | ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions |
title_full | ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions |
title_fullStr | ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions |
title_full_unstemmed | ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions |
title_short | ABHD5—A Regulator of Lipid Metabolism Essential for Diverse Cellular Functions |
title_sort | abhd5—a regulator of lipid metabolism essential for diverse cellular functions |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9694394/ https://www.ncbi.nlm.nih.gov/pubmed/36355098 http://dx.doi.org/10.3390/metabo12111015 |
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