CPT2 Deficiency Modeled in Zebrafish: Abnormal Neural Development, Electrical Activity, Behavior, and Schizophrenia-Related Gene Expression DOI Creative Commons

Carly E. Baker,

Aaron G. Marta,

Nathan D. Zimmerman

et al.

Biomolecules, Journal Year: 2024, Volume and Issue: 14(8), P. 914 - 914

Published: July 26, 2024

Carnitine palmitoyltransferase 2 (CPT2) is an inner mitochondrial membrane protein of the carnitine shuttle and involved in beta-oxidation long chain fatty acids. Beta-oxidation provides alternative pathway energy production during early development starvation. CPT2 deficiency a genetic disorder that we recently showed can be associated with schizophrenia. We hypothesize brain causes transcriptional, structural, functional abnormalities may contribute to CNS environment susceptible emergence To investigate effect on vertebrate function, was knocked down zebrafish model system. knockdown resulted abnormal lipid utilization deposition, reduction body size, development. Axonal projections, neurotransmitter synthesis, electrical hyperactivity, swimming behavior were disrupted zebrafish. RT-qPCR analyses significant increases expression schizophrenia-associated genes compared control Taken together, these data demonstrate are useful for studying importance function. This study also presents novel findings linking regulation schizophrenia neurodegenerative disease-associated genes.

Language: Английский

Disorders of fatty acid homeostasis DOI Creative Commons
Frédéric M. Vaz, Sacha Ferdinandusse, Gajja S. Salomons

et al.

Journal of Inherited Metabolic Disease, Journal Year: 2024, Volume and Issue: 48(1)

Published: May 1, 2024

Abstract Humans derive fatty acids (FA) from exogenous dietary sources and/or endogenous synthesis acetyl‐CoA, although some FA are solely derived (“essential FA”). Once inside cells, may undergo a wide variety of different modifications, which include their activation to corresponding CoA ester, the introduction double bonds, 2‐ and ω‐hydroxylation chain elongation, thereby generating cellular pool can be used for more complex lipids. The biological properties lipids very much determined by molecular composition in terms incorporated into these lipid species. This immediately explains existence range genetic diseases man, often with severe clinical consequences caused variants one many genes coding enzymes responsible modifications. It is purpose this review describe current state knowledge about homeostasis involved. includes disorders activation, desaturation, ω‐hydroxylation, but also breakdown, including peroxisomal mitochondrial α‐ β‐oxidation.

Language: Английский

Citations

5
The Role of Linoleic Acid in Skin and Hair Health: A Review DOI Open Access
Xi Wang, Yan Jia, Huaming He

et al.

International Journal of Molecular Sciences, Journal Year: 2024, Volume and Issue: 26(1), P. 246 - 246

Published: Dec. 30, 2024

Lipids are intimately associated with skin condition. This review aims to discuss the function of linoleic acid (LA, 18:2, ω-6), an essential fatty acid, in health and hair growth. In skin, LA can be metabolized into ω-6 unsaturated oxidized derivatives incorporated complex lipid molecules, including ω-hydroxy-ceramides. Previous research has revealed that diseases acne, atopic dermatitis psoriasis disordered metabolism. Studies based on animal or cell models suggest LA-rich vegetable oils, topically applied, exhibit diverse biological activities, repair barrier, promotion wound healing, whitening, photoprotection, anti-inflammatory effects stimulation Moreover, underlying mechanisms LA’s beneficial summarized. Further correlation metabolism disorders, a deeper exploration management more investigations its clinical application required enhance understanding utilization cosmetics pharmaceuticals.

Language: Английский

Citations

4
The ELOVL proteins: Very and ultra long-chain fatty acids at the crossroads between metabolic and neurodegenerative disorders DOI Creative Commons
Enza Ferrero, Frédéric M. Vaz, David Cheillan

et al.

Molecular Genetics and Metabolism, Journal Year: 2025, Volume and Issue: 144(3), P. 109050 - 109050

Published: Feb. 4, 2025

Language: Английский

Citations

0
Interorganelle phospholipid communication, a house not so divided DOI
Richard G. Lee, Danielle L. Rudler, Oliver Rackham

et al.

Trends in Endocrinology and Metabolism, Journal Year: 2024, Volume and Issue: 35(10), P. 872 - 883

Published: July 6, 2024

Language: Английский

Citations

0
CPT2 Deficiency Modeled in Zebrafish: Abnormal Neural Development, Electrical Activity, Behavior, and Schizophrenia-Related Gene Expression DOI Creative Commons

Carly E. Baker,

Aaron G. Marta,

Nathan D. Zimmerman

et al.

Biomolecules, Journal Year: 2024, Volume and Issue: 14(8), P. 914 - 914

Published: July 26, 2024

Carnitine palmitoyltransferase 2 (CPT2) is an inner mitochondrial membrane protein of the carnitine shuttle and involved in beta-oxidation long chain fatty acids. Beta-oxidation provides alternative pathway energy production during early development starvation. CPT2 deficiency a genetic disorder that we recently showed can be associated with schizophrenia. We hypothesize brain causes transcriptional, structural, functional abnormalities may contribute to CNS environment susceptible emergence To investigate effect on vertebrate function, was knocked down zebrafish model system. knockdown resulted abnormal lipid utilization deposition, reduction body size, development. Axonal projections, neurotransmitter synthesis, electrical hyperactivity, swimming behavior were disrupted zebrafish. RT-qPCR analyses significant increases expression schizophrenia-associated genes compared control Taken together, these data demonstrate are useful for studying importance function. This study also presents novel findings linking regulation schizophrenia neurodegenerative disease-associated genes.

Language: Английский

Citations

0