Neutral Lipid Storage Diseases as Cellular Model to Study Lipid Droplet Function

Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the and in the genes, respectively. These genes encode the adipose triglyceride lipase (ATGL) and α-β hydrolase domain 5 (ABHD5) proteins, which play key role...

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Published inCells (Basel, Switzerland) Vol. 8; no. 2; p. 187
Main Authors Missaglia, Sara, Coleman, Rosalind A, Mordente, Alvaro, Tavian, Daniela
Format Journal Article
LanguageEnglish
Published Switzerland MDPI AG 21.02.2019
MDPI
Subjects
1-Acylglycerol-3-Phosphate O-Acyltransferase - chemistry
1-Acylglycerol-3-Phosphate O-Acyltransferase - metabolism
ABHD5
Adipocytes
Animals
Biosynthesis
Cardiac muscle
cardiomyopathy
Cardiovascular disease
Cell culture
Diabetes
Energy balance
Energy metabolism
Esters
Fibroblasts
Gene expression
Homeostasis
Humans
Hydrolase
Ichthyosis
induced pluripotent stem cells
Insulin resistance
Jordans’ anomaly
lipid droplet
Lipid Droplets - metabolism
Lipid Droplets - ultrastructure
Lipid metabolism
Lipid Metabolism, Inborn Errors - metabolism
Lipids
liver steatosis
Mammals
Metabolism
Models, Biological
Muscles - pathology
Muscles - ultrastructure
Mutation
Myopathy
NLSD
Organelles
PNPLA2
Proteins
Review
Storage diseases
Structure-function relationships
Vitamin A
lipid droplet
fibroblasts
myopathy
lipid metabolism
liver steatosis
Jordans’ anomaly
induced pluripotent stem cells
ABHD5
NLSD
cardiomyopathy
ichthyosis
PNPLA2
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Abstract Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the and in the genes, respectively. These genes encode the adipose triglyceride lipase (ATGL) and α-β hydrolase domain 5 (ABHD5) proteins, which play key roles in the function of lipid droplets (LDs). LDs, the main cellular storage sites of triacylglycerols and sterol esters, are highly dynamic organelles. Indeed, LDs are critical for both lipid metabolism and energy homeostasis. Partial or total or knockdown is characteristic of the cells of NLSD patients; thus, these cells are natural models with which one can unravel LD function. In this review we firstly summarize genetic and clinical data collected from NLSD patients, focusing particularly on muscle, skin, heart, and liver damage due to impaired LD function. Then, we discuss how NLSD cells were used to investigate and expand the current structural and functional knowledge of LDs.
AbstractList Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the PNPLA2 and in the ABHD5/CGI58 genes, respectively. These genes encode the adipose triglyceride lipase (ATGL) and α-β hydrolase domain 5 (ABHD5) proteins, which play key roles in the function of lipid droplets (LDs). LDs, the main cellular storage sites of triacylglycerols and sterol esters, are highly dynamic organelles. Indeed, LDs are critical for both lipid metabolism and energy homeostasis. Partial or total PNPLA2 or ABHD5/CGI58 knockdown is characteristic of the cells of NLSD patients; thus, these cells are natural models with which one can unravel LD function. In this review we firstly summarize genetic and clinical data collected from NLSD patients, focusing particularly on muscle, skin, heart, and liver damage due to impaired LD function. Then, we discuss how NLSD cells were used to investigate and expand the current structural and functional knowledge of LDs.
Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the PNPLA2 and in the ABHD5/CGI58 genes, respectively. These genes encode the adipose triglyceride lipase (ATGL) and α-β hydrolase domain 5 (ABHD5) proteins, which play key roles in the function of lipid droplets (LDs). LDs, the main cellular storage sites of triacylglycerols and sterol esters, are highly dynamic organelles. Indeed, LDs are critical for both lipid metabolism and energy homeostasis. Partial or total PNPLA2 or ABHD5/CGI58 knockdown is characteristic of the cells of NLSD patients; thus, these cells are natural models with which one can unravel LD function. In this review we firstly summarize genetic and clinical data collected from NLSD patients, focusing particularly on muscle, skin, heart, and liver damage due to impaired LD function. Then, we discuss how NLSD cells were used to investigate and expand the current structural and functional knowledge of LDs.
Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the and in the genes, respectively. These genes encode the adipose triglyceride lipase (ATGL) and α-β hydrolase domain 5 (ABHD5) proteins, which play key roles in the function of lipid droplets (LDs). LDs, the main cellular storage sites of triacylglycerols and sterol esters, are highly dynamic organelles. Indeed, LDs are critical for both lipid metabolism and energy homeostasis. Partial or total or knockdown is characteristic of the cells of NLSD patients; thus, these cells are natural models with which one can unravel LD function. In this review we firstly summarize genetic and clinical data collected from NLSD patients, focusing particularly on muscle, skin, heart, and liver damage due to impaired LD function. Then, we discuss how NLSD cells were used to investigate and expand the current structural and functional knowledge of LDs.
Author Missaglia, Sara
Mordente, Alvaro
Coleman, Rosalind A
Tavian, Daniela
AuthorAffiliation 5 Facoltà di Scienze della Formazione, Università Cattolica del Sacro Cuore, 20123 Milano, Italy
3 Department of Nutrition, University of North Carolina, Chapel Hill, NC 27599, USA; rcoleman@unc.edu
4 Dipartimento di Scienze di Laboratorio ed Infettivologiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, Italy; Alvaro.Mordente@unicatt.it
1 Laboratory of Cellular Biochemistry and Molecular Biology, CRIBENS, Università Cattolica del Sacro Cuore, 20145 Milan, Italy; sara.missaglia@unicatt.it
2 Psychology Department, Università Cattolica del Sacro Cuore, 20123 Milan, Italy
AuthorAffiliation_xml – name: 1 Laboratory of Cellular Biochemistry and Molecular Biology, CRIBENS, Università Cattolica del Sacro Cuore, 20145 Milan, Italy; sara.missaglia@unicatt.it
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– name: 5 Facoltà di Scienze della Formazione, Università Cattolica del Sacro Cuore, 20123 Milano, Italy
– name: 2 Psychology Department, Università Cattolica del Sacro Cuore, 20123 Milan, Italy
– name: 4 Dipartimento di Scienze di Laboratorio ed Infettivologiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, Italy; Alvaro.Mordente@unicatt.it
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  orcidid: 0000-0001-6551-6698
  surname: Missaglia
  fullname: Missaglia, Sara
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  organization: Psychology Department, Università Cattolica del Sacro Cuore, 20123 Milan, Italy. sara.missaglia@unicatt.it
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  orcidid: 0000-0003-4379-3824
  surname: Coleman
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  organization: Department of Nutrition, University of North Carolina, Chapel Hill, NC 27599, USA. rcoleman@unc.edu
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  givenname: Alvaro
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  fullname: Mordente, Alvaro
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  email: daniela.tavian@unicatt.it, daniela.tavian@unicatt.it
  organization: Psychology Department, Università Cattolica del Sacro Cuore, 20123 Milan, Italy. daniela.tavian@unicatt.it
BackLink https://www.ncbi.nlm.nih.gov/pubmed/30795549$$D View this record in MEDLINE/PubMed
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Issue 2
Keywords lipid droplet
fibroblasts
myopathy
lipid metabolism
liver steatosis
Jordans’ anomaly
induced pluripotent stem cells
ABHD5
NLSD
cardiomyopathy
ichthyosis
PNPLA2
Language English
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Snippet Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the and in the...
Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the PNPLA2 and in...
Neutral lipid storage disease with myopathy (NLSDM) and with ichthyosis (NLSDI) are rare autosomal recessive disorders caused by mutations in the PNPLA2 and in...
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SubjectTerms 1-Acylglycerol-3-Phosphate O-Acyltransferase - chemistry
1-Acylglycerol-3-Phosphate O-Acyltransferase - metabolism
ABHD5
Adipocytes
Animals
Biosynthesis
Cardiac muscle
cardiomyopathy
Cardiovascular disease
Cell culture
Diabetes
Energy balance
Energy metabolism
Esters
Fibroblasts
Gene expression
Homeostasis
Humans
Hydrolase
Ichthyosis
induced pluripotent stem cells
Insulin resistance
Jordans’ anomaly
lipid droplet
Lipid Droplets - metabolism
Lipid Droplets - ultrastructure
Lipid metabolism
Lipid Metabolism, Inborn Errors - metabolism
Lipids
liver steatosis
Mammals
Metabolism
Models, Biological
Muscles - pathology
Muscles - ultrastructure
Mutation
Myopathy
NLSD
Organelles
PNPLA2
Proteins
Review
Storage diseases
Structure-function relationships
Vitamin A
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Title Neutral Lipid Storage Diseases as Cellular Model to Study Lipid Droplet Function
URI https://www.ncbi.nlm.nih.gov/pubmed/30795549
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