Crystal structure of the rice acyl‐CoA‐binding protein OsACBP2 in complex with C18:3‐CoA reveals a novel pattern of binding to acyl‐CoA esters
Sign Up to like & getrecommendations! Published in 2020 at "FEBS Letters"
DOI: 10.1002/1873-3468.13923
Abstract: Acyl‐CoA‐binding proteins (ACBPs) are a family of proteins that bind acyl‐CoA esters at a conserved acyl‐CoA‐binding domain. ACBPs maintain intracellular acyl‐CoA pools to regulate lipid metabolism. Here, we report on the structure of rice OsACBP2… read more here.
Keywords: osacbp2; c18 coa; acyl coa; coa ... See more keywords
The acyl‐CoA‐binding protein Acb1 regulates mitochondria, lipid droplets, and cell proliferation
Sign Up to like & getrecommendations! Published in 2022 at "FEBS Letters"
DOI: 10.1002/1873-3468.14415
Abstract: Mitochondria are involved in many cellular activities, including energy metabolism and biosynthesis of nucleotides, fatty acids and amino acids. Mitochondrial morphology is a key factor in dictating mitochondrial functions. Here, we report that the acyl‐CoA‐binding… read more here.
Keywords: binding protein; coa binding; lipid droplets; acb1 regulates ... See more keywords
Acyl‐CoA synthetase short‐chain family member 2 (ACSS2) is regulated by SREBP‐1 and plays a role in fatty acid synthesis in caprine mammary epithelial cells
Sign Up to like & getrecommendations! Published in 2018 at "Journal of Cellular Physiology"
DOI: 10.1002/jcp.25954
Abstract: Sterol regulatory element binding protein 1 (SREBP‐1) is well‐known as the master regulator of lipogenesis in rodents. Acyl‐CoA synthetase short‐chain family member 2 (ACSS2) plays a key role in lipogenesis by synthesizing acetyl‐CoA from acetate… read more here.
Keywords: fatty acid; lipogenesis; acyl coa; acss2 ... See more keywords
Prediction of disease severity in multiple acyl‐CoA dehydrogenase deficiency: A retrospective and laboratory cohort study
Sign Up to like & getrecommendations! Published in 2019 at "Journal of Inherited Metabolic Disease"
DOI: 10.1002/jimd.12147
Abstract: Multiple acyl‐CoA dehydrogenase deficiency (MADD) is an ultra‐rare inborn error of mitochondrial fatty acid oxidation (FAO) and amino acid metabolism. Individual phenotypes and treatment response can vary markedly. We aimed to identify markers that predict… read more here.
Keywords: madd ds3; disease severity; multiple acyl; acyl coa ... See more keywords
Enantiomer‐specific pharmacokinetics of D,L‐3‐hydroxybutyrate: Implications for the treatment of multiple acyl‐CoA dehydrogenase deficiency
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Inherited Metabolic Disease"
DOI: 10.1002/jimd.12365
Abstract: D,L‐3‐hydroxybutyrate (D,L‐3‐HB, a ketone body) treatment has been described in several inborn errors of metabolism, including multiple acyl‐CoA dehydrogenase deficiency (MADD; glutaric aciduria type II). We aimed to improve the understanding of enantiomer‐specific pharmacokinetics of… read more here.
Keywords: coa dehydrogenase; specific pharmacokinetics; acyl coa; enantiomer specific ... See more keywords
Plasma lipidomics analysis reveals altered profile of triglycerides and phospholipids in children with Medium‐Chain Acyl‐CoA dehydrogenase deficiency
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DOI: 10.1002/jimd.12718
Abstract: Medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD) is the most prevalent mitochondrial fatty acid β‐oxidation disorder. In this study, we assessed the variability of the lipid profile in MCADD by analysing plasma samples obtained from 25 children… read more here.
Keywords: medium chain; analysis; acyl coa; chain acyl ... See more keywords
Growing evidence for a role for acyl‐CoA synthetase 1 in immunometabolism
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DOI: 10.1002/jlb.3ce0519-154r
Abstract: Discussion on the role of acyl-CoA synthetase 1 in immunometabolism. read more here.
Keywords: synthetase immunometabolism; acyl coa; coa synthetase; role acyl ... See more keywords
Characterization of Variants of Uncertain Significance in ACADVL Gene From a Very–Long‐Chain Acyl‐CoA Dehydrogenase Deficiency Patient
Sign Up to like & getrecommendations! Published in 2025 at "Molecular Genetics & Genomic Medicine"
DOI: 10.1002/mgg3.70120
Abstract: Very–long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD) is a rare disorder of long‐chain mitochondrial fatty acid oxidation (FAO) caused by biallelic mutations in the acyl‐CoA dehydrogenase very–long‐chain (ACADVL) gene with autosomal recessive (AR) inheritance. Currently, the ACADVL… read more here.
Keywords: acyl coa; long chain; coa dehydrogenase; acadvl gene ... See more keywords
Comment on: “Multiple acyl‑CoA dehydrogenase deficiency in elderly carriers”
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DOI: 10.1007/s00415-020-09752-0
Abstract: We read the paper “Multiple acyl‐CoA dehydrogenase defi‐ ciency [MADD] in elderly carriers” by Macchione et al. with great interest, where they describe two elderly individuals, each with only one heterozygous variant in ETFDH gene… read more here.
Keywords: coa dehydrogenase; variant; elderly carriers; acyl coa ... See more keywords
Studying the topology of peroxisomal acyl-CoA synthetases using self-assembling split sfGFP
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DOI: 10.1007/s00418-023-02257-7
Abstract: Peroxisomes are membrane-bounded organelles that contain enzymes involved in multiple lipid metabolic pathways. Several of these pathways require (re-)activation of fatty acids to coenzyme A (CoA) esters by acyl-CoA synthetases, which may take place inside… read more here.
Keywords: assembling split; acyl coa; coa synthetases; topology ... See more keywords
Analyses of the genetic diversity and protein expression variation of the acyl: CoA medium-chain ligases, ACSM2A and ACSM2B
Sign Up to like & getrecommendations! Published in 2018 at "Molecular Genetics and Genomics"
DOI: 10.1007/s00438-018-1460-3
Abstract: Benzoate (found in milk and widely used as preservative), salicylate (present in fruits and the active component of aspirin), dietary polyphenols produced by gut microbiota, metabolites from organic acidemias, and medium-chain fatty acids (MCFAs) are… read more here.
Keywords: protein expression; medium chain; acsm2a acsm2b; acyl coa ... See more keywords