TECR

TECR (Trans-2,3-enoyl-CoA reductase) is a human gene that encodes for an enzyme involved in the final step of the mitochondrial fatty acid synthesis (FAS) pathway. This pathway is crucial for the production of lipids that are essential for the normal function of mitochondria, the energy-producing organelles in cells.

Function

TECR is an enzyme that catalyzes the reduction of trans-2,3-enoyl-CoA to acyl-CoA with chain lengths from C6 to C16 in the presence of NADPH. This is the final step in the mitochondrial fatty acid synthesis (FAS) pathway. The FAS pathway is responsible for the production of lipids that are essential for the normal function of mitochondria.

Clinical significance

Mutations in the TECR gene have been associated with non-syndromic mental retardation and leukodystrophy, a rare genetic disorder affecting the white matter of the brain.

See also

• Fatty acid synthesis
• Mitochondria
• Leukodystrophy

References

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