Related Experiment Video
Updated: Aug 7, 2026

Establishment of an Extracellular Acidic pH Culture System
Published on: November 19, 2017
Tricarboxylic acid cycle dysfunction as a cause of human diseases and tumor formation
Jean-Jacques Brière1, Judith Favier, Anne-Paule Gimenez-Roqueplo
1Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 676, Hôpital Robert Debré, Paris.
Abstract:
A renewed interest in tricarboxylic acid cycle enzymopathies has resulted from the report that, in addition to devastating encephalopathies, these can result in various types of tumors in human. We first review the major features of the cycle that may underlie this surprising variety of clinical features. After discussing the rare cases of encephalopathies associated with specific deficiencies of some of the tricarboxylic acid cycle enzyme, we finally examine the mechanism possibly causing tumor/cancer formation in the cases of mutations affecting fumarase or succinate dehydrogenase genes.
Related Concept Videos
Loss of Carboxy Group as CO2: Decarboxylation of β-Ketoacids
Allosteric Proteins-ATCase
Aspartate transcarbamoylase (ATCase) is a cytosolic enzyme that catalyzes the condensation of L-aspartate and carbamoyl phosphate to N-carbamoyl-L-aspartate. This reaction is the first step in pyrimidine biosynthesis. UTP and CTP, the end products of the pyrimidine synthesis pathway,...
Electron Transport Chain: Complex I and II
ROS generation is regulated and maintained at moderate levels necessary...
Mutagenicity and Carcinogenicity
Overview of Lipid Metabolism
Lipolysis: The Breakdown of Lipids:
Lipolysis is the process of breaking down lipids, particularly triglycerides, into glycerol and fatty acids. This process typically occurs in the adipose tissue and is triggered by various hormones, including glucagon and...
Adaptive Mechanisms in Cancer Cells
Some of the advantages that cancer cells have on normal cells include - enhanced ability to divide without terminally differentiating, induce new blood vessel formation,...

