Zeta Fumarate Hydratase Antibody. Zeta’s recombinant rabbit antibody recognizes Fumarate Hydratase (FH), also known as fumarase, an enzyme that plays a critical role in the Krebs cycle. Fumarate Hydratase catalyzes the reversible hydration of fumarate to malate. By immunohistochemistry, normal tissues with rich mitochondria, such as the kidney and liver, are strongly positive for Fumarate Hydratase (FH). Germline mutations in the Fumarate Hydratase gene cause Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC). This genetic syndrome predisposes individuals to develop benign smooth muscle tumors (leiomyomas) and an aggressive form of kidney cancer (renal cell carcinoma).
Fumarate Hydratase deficient pheochromocytomas (PCCs) and paragangliomas (PGLs) are underrecognized but can be identified by IHC. Fumarate Hydratase (FH) deficient PCC/PGL are strongly associated with germline missense mutations but are infrequently associated with leiomyoma or RCC, suggesting there may be a genotype-phenotype correlation. Fumarate Hydratase (FH) deficient PCC/PGL may have a higher metastatic risk (A Clinicopathologic and Molecular Analysis of Fumarate Hydratase–deficient Pheochromocytoma and Paraganglioma – PMC).
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