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Updated: Oct 10, 2025

Quantification of Coenzyme A in Cells and Tissues
Published on: September 27, 2019
Emerging Role of TCA Cycle-Related Enzymes in Human Diseases
Woojin Kang1, Miki Suzuki1, Takako Saito2
1Department of Reproductive Biology, National Research Institute for Child Health and Development, Tokyo 157-8535, Japan.
Abstract:
The tricarboxylic acid (TCA) cycle is the main source of cellular energy and participates in many metabolic pathways in cells. Recent reports indicate that dysfunction of TCA cycle-related enzymes causes human diseases, such as neurometabolic disorders and tumors, have attracted increasing interest in their unexplained roles. The diseases which develop as a consequence of loss or dysfunction of TCA cycle-related enzymes are distinct, suggesting that each enzyme has a unique function. This review aims to provide a comprehensive overview of the relationship between each TCA cycle-related enzyme and human diseases. We also discuss their functions in the context of both mitochondrial and extra-mitochondrial (or cytoplasmic) enzymes.
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