Carnitine palmitoyltransferase I

Carnitine palmitoyltransferase I (pronounced: kar-ni-teen pal-mi-toy-il-trans-fer-ase one), often abbreviated as CPT1, is an enzyme that plays a crucial role in the metabolism of fatty acids in the body.

Etymology

The term "Carnitine" is derived from the Latin carnus, meaning "flesh", as it was first isolated from meat. "Palmitoyltransferase" is a compound word formed from "palmitoyl", referring to the fatty acid palmitic acid, and "transferase", a type of enzyme that helps transfer a functional group from one molecule to another.

Function

CPT1 is responsible for the transfer of long-chain fatty acids from the cytosol into the mitochondria, a process known as fatty acid oxidation. This is a critical step in the production of energy in cells.

Types

There are three known isoforms of CPT1: CPT1A, CPT1B, and CPT1C, each with different tissue distribution and regulatory properties.

Clinical significance

Mutations in the genes encoding CPT1 can lead to Carnitine palmitoyltransferase I deficiency, a rare genetic disorder that prevents the body from converting certain types of fats into energy.

Related terms

• Fatty acid oxidation
• Carnitine palmitoyltransferase II
• Carnitine palmitoyltransferase I deficiency
• Mitochondria
• Enzyme

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