Fumarase Specific Activity Assay Kit

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Fumarase sometimes referred to as fumarate hydratase is an enzyme involved in the Krebs cycle. Its mechanical function involves the reversible hydration/dehydration of fumarate to malate. The enzyme has a molecular mass around 50 kilodaltons. Fumarase expression occurs in the mitochondria of eukaryotic cells and it also appears in the cytosol contributing to the compartmentalization of its enzymatic activity.

Biological function summary

Fumarase plays a critical role in cellular respiration by facilitating the conversion of fumarate to malate within the tricarboxylic acid (TCA) cycle. This enzyme does not operate as part of a larger protein complex but its activity is essential for maintaining the flow of carbon through the TCA cycle which is integral to energy production. Its activity is upregulated when cells require increased ATP generation.

Pathways

Fumarase participates prominently in the TCA cycle a vital pathway for aerobic respiration where it interconverts fumarate and malate. The enzyme supports cellular energy production and this is closely linked with other TCA cycle enzymes like isocitrate dehydrogenase and alpha-ketoglutarate dehydrogenase which further facilitate efficient energy conversion.

Associated diseases and disorders

Fumarase deficiency leads to severe neurological impairment often manifesting as encephalopathy. This deficiency occurs due to mutations in the FH gene which encodes for fumarase. Additionally a reduction in fumarase activity associates with fumarase-related hereditary leiomyomatosis and renal cell cancer (HLRCC) linking fumarase with altered metabolic states in tumorigenesis. Dysregulation within this context often involves interactions with metabolically similar enzymes complicating the metabolic landscape and contributing to oncogenic processes.