Overlapping and Distinct Features of Cardiac Pathology in Inherited Human and Murine Ether Lipid Deficiency.
cardiac disease
ether lipid
left ventricular hypertrophy
peroxisome
plasmalogen
rhizomelic chondrodysplasia punctata
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
18 Jan 2023
18 Jan 2023
Historique:
received:
28
11
2022
revised:
10
01
2023
accepted:
11
01
2023
entrez:
11
2
2023
pubmed:
12
2
2023
medline:
15
2
2023
Statut:
epublish
Résumé
Inherited deficiency in ether lipids, a subgroup of glycerophospholipids with unique biochemical and biophysical properties, evokes severe symptoms in humans resulting in a multi-organ syndrome. Mouse models with defects in ether lipid biosynthesis have widely been used to understand the pathophysiology of human disease and to study the roles of ether lipids in various cell types and tissues. However, little is known about the function of these lipids in cardiac tissue. Previous studies included case reports of cardiac defects in ether-lipid-deficient patients, but a systematic analysis of the impact of ether lipid deficiency on the mammalian heart is still missing. Here, we utilize a mouse model of complete ether lipid deficiency (
Identifiants
pubmed: 36768204
pii: ijms24031884
doi: 10.3390/ijms24031884
pmc: PMC9914995
pii:
doi:
Substances chimiques
Ether
0F5N573A2Y
Ethers
0
Ethyl Ethers
0
Plasmalogens
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : FWF Austrian Science Fund
ID : P24843-B24
Organisme : FWF Austrian Science Fund
ID : I2738-B26
Organisme : FWF Austrian Science Fund
ID : P31082-B21
Organisme : FWF Austrian Science Fund
ID : P34723
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