Target intelligence / Profile preview

Mitochondrial Inner Membrane Fatty Acid Transport System (Mitochondrial FA Transport)

Target
Mitochondrial FA Transport
Molecular classification
Enzyme, Transporter
01

Overview

The mitochondrial inner membrane fatty acid transport system, primarily the carnitine shuttle, is essential for transporting long-chain fatty acids from the cytosol into the mitochondrial matrix for beta-oxidation and ATP production. It involves CPT1 (outer membrane), carnitine-acylcarnitine translocase (inner membrane), and CPT2 (matrix side). Defects can lead to fatty acid oxidation disorders.

Other names
Carnitine Shuttle SystemFatty Acid Transport Across Mitochondrial MembraneCPT1/CPT2/CAC SystemCarnitine Palmitoyltransferase System
02

Mechanism of action

N/A

03

Biological functions

Fatty acid transportBeta-oxidationEnergy productionMitochondrial metabolism
04

Disease associations

Fatty acid oxidation disordersHypoketotic hypoglycemiaMuscle weaknessRhabdomyolysisMetabolic myopathies
05

Safety considerations

Impaired fatty acid oxidationHypoglycemiaMuscle damage

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