ECHS1


Description

ECHS1, also known as enoyl-CoA hydratase, short-chain, is a crucial enzyme involved in the breakdown of fatty acids. It plays a pivotal role in the beta-oxidation pathway, catalyzing the hydration of trans-2-enoyl-CoA intermediates, a critical step in the process. This enzyme is predominantly expressed in the liver and skeletal muscles, reflecting its significant role in energy production from fat. ECHS1 deficiency, a rare genetic disorder, can lead to a buildup of fatty acids, impacting energy metabolism and potentially causing various health issues.

Associated Diseases

Did you know?

ECHS1 is one of the enzymes responsible for the characteristic odor of sweat in humans, contributing to the unique scent of each individual.


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