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Fatty Acid Mitochondrial Transport System (Carnitine Shuttle) (Carnitine Shuttle)
- Target
- Carnitine Shuttle
- Molecular classification
- Enzyme System, Transporter System
01
Overview
The fatty acid mitochondrial transport system, commonly known as the carnitine shuttle, is a critical biochemical pathway that enables the transport of long-chain fatty acids from the cytosol into the mitochondrial matrix for β-oxidation and subsequent energy production. It involves CPT I, carnitine-acylcarnitine translocase, and CPT II.
- Other names
- Carnitine Palmitoyltransferase SystemFatty Acid Transport SystemCPT System
02
Mechanism of action
The carnitine shuttle transports long-chain fatty acids from the cytosol into the mitochondrial matrix for beta-oxidation.
03
Biological functions
Fatty Acid TransportBeta-OxidationEnergy ProductionLipid Metabolism
04
Disease associations
Carnitine DeficiencyFatty Acid Oxidation DisordersHypoketotic HypoglycemiaMuscle WeaknessMyopathyRhabdomyolysis
05
Safety considerations
Inhibition of CPT I can lead to accumulation of fatty acids in the cytosol.Defects in any component can lead to metabolic disorders.
06
Interacting drugs
Malonyl-CoA
1 more in the full profile.
07
Biomarkers
Acylcarnitine levelsFatty acid levelsCarnitine levels
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