Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Schratter, Margarita, Lass, Achim, Radner, Franz P. W.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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
_version_ 1784837789063315456
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
work_keys_str_mv AT schrattermargarita abhd5aregulatoroflipidmetabolismessentialfordiversecellularfunctions
AT lassachim abhd5aregulatoroflipidmetabolismessentialfordiversecellularfunctions
AT radnerfranzpw abhd5aregulatoroflipidmetabolismessentialfordiversecellularfunctions