CARNITINE ACYLCARNITINE TRANSLOCASE Meaning and
Definition
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Carnitine Acylcarnitine Translocase (CACT) is an enzyme that plays a crucial role in the transportation of long-chain fatty acids across the mitochondrial membrane. This translocase is responsible for the import of fatty acids into the mitochondria, where they undergo beta-oxidation, a process that generates energy.
CACT is a transmembrane protein located in the inner mitochondrial membrane and acts as a carrier molecule. It facilitates the movement of long-chain fatty acylcarnitines, which are formed when fatty acids are conjugated to carnitine, from the cytosol into the mitochondrial matrix. It accomplishes this by exchanging intramitochondrial free carnitine with the long-chain fatty acylcarnitines across the membrane.
The translocation of fatty acids is essential for their subsequent metabolism and energy production. CACT ensures that the long-chain fatty acylcarnitines are delivered to the mitochondrial matrix, where they can be further processed to release energy through beta-oxidation. This process is particularly important during periods of increased energy demand, such as exercise or fasting, when fatty acids serve as a primary energy source.
Defects in CACT function can lead to a rare genetic disorder called CACT deficiency, which is characterized by impaired fatty acid oxidation. The accumulation of acylcarnitines due to CACT deficiency can result in various metabolic disturbances, including muscle weakness, hypoglycemia, liver dysfunction, and potentially life-threatening metabolic crises. Treatment typically involves dietary modifications, such as avoiding fasting and supplementation with medium-chain triglycerides as an alternative fuel source.
Common Misspellings for CARNITINE ACYLCARNITINE TRANSLOCASE
- xarnitine acylcarnitine translocase
- varnitine acylcarnitine translocase
- farnitine acylcarnitine translocase
- darnitine acylcarnitine translocase
- czrnitine acylcarnitine translocase
- csrnitine acylcarnitine translocase
- cwrnitine acylcarnitine translocase
- cqrnitine acylcarnitine translocase
- caenitine acylcarnitine translocase
- cadnitine acylcarnitine translocase
- cafnitine acylcarnitine translocase
- catnitine acylcarnitine translocase
- ca5nitine acylcarnitine translocase
- ca4nitine acylcarnitine translocase
- carbitine acylcarnitine translocase
- carmitine acylcarnitine translocase
- carjitine acylcarnitine translocase
- carhitine acylcarnitine translocase
- carnutine acylcarnitine translocase
- carnjtine acylcarnitine translocase
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