FH(Fumarate hydratase) also known as Fumarate is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. FH plays a significant role in the Krebs cycle by catalyzing the reversible hydration of fumaric acid to produce l-malic acid. This enzymatic activity is vital for cellular respiration and energy production, highlighting the importance of FH in metabolic processes. Notably, germline mutations in the FH gene are linked to a predisposition for renal defects, including multiple cutaneous and uterine leiomyoma syndrome, as well as an increased risk of renal and smooth muscle tumors, although they do not correlate with prostate cancer. Additionally, FH deficiency has been associated with neurological complications, such as seizures, stemming from prenatal brain dysgenesis.