24
Jul
Fatty Acyl Coa Synthetase Deficiency. Long-chain acyl-CoA synthetase isoform 1 ACSL1 deficiency in the heart activated mTORC1 thereby inhibiting autophagy and increasing the number of damaged mitochondria. Long-chain acyl-CoA synthetase 1 ACSL1 contributes more than 90 of total cardiac ACSL activity but its role in phospholipid synthesis has not been determined. ACSL4 belonging to the ACSL family shows a preferential use of arachidonic acid AA as its substrate and plays a role in the remodeling of AA-containing phospholipids by incorporating free AA. Our data suggest that ACSL1 is required to synthesize the acyl-CoAs that are oxidized by the heart and that without ACSL1 diminished fatty acid FA oxidation and compensatory catabolism of glucose and amino acids lead to mTOR activation and cardiac hypertrophy without lipid accumulation or immediate cardiac dysfunction.
This reaction is similar. Our data suggest that ACSL1 is required to synthesize the acyl-CoAs that are oxidized by the heart and that without ACSL1 diminished fatty acid FA oxidation and compensatory catabolism of glucose and amino acids lead to mTOR activation and cardiac hypertrophy without lipid accumulation or immediate cardiac dysfunction. Mice with an inducible knockout of ACSL1 Acsl1T have impaired cardiac fatty. The family of acyl-CoA synthetase enzymes ACSL activates fatty acids within cells to generate long chain fatty acyl CoA FACoA. Long-chain acyl-CoA synthetase 1 ACSL1 contributes more than 90 of total cardiac ACSL activity but its role in phospholipid synthesis has not been determined. These mutations lead to a shortage deficiency of an enzyme known as short-chain acyl-CoA dehydrogenase which is involved in the breakdown of short-chain fatty acids.
Long-chain acyl-coenzyme A synthetases ACSLs are a family of enzymes that convert free long-chain fatty acids into their acyl-coenzyme A CoA forms.
Our data suggest that ACSL1 is required to synthesize the acyl-CoAs that are oxidized by the heart and that without ACSL1 diminished fatty acid FA oxidation and compensatory catabolism of. Acyl-CoA synthetase 1 deficiency alters cardiolipin species and impairs mitochondrial function ACSL1 is a programmable mediator of insulin sensitivity and cellular lipid content. 2011 Mouse cardiac acyl coenzyme a synthetase 1 deficiency impairs fatty acid oxidation and induces cardiac hypertrophy. Department of Genetics College of Medicine and Health Sciences Sultan Qaboos University Muscat Oman. The family of acyl-CoA synthetase enzymes ACSL activates fatty acids within cells to generate long chain fatty acyl CoA FACoA. When not enough of this enzyme is present excessive amounts of fatty acids and ammonia accumulate in the body.