Fumarase Antibody
Description
Product Specs
Product Science Overview
Fumarate Hydratase (FH), also known as fumarase, is an enzyme that plays a crucial role in the tricarboxylic acid (TCA) cycle, also known as the Krebs cycle. This enzyme catalyzes the reversible hydration of fumarate to L-malate . FH exists in two forms: a cytosolic form and a mitochondrial form. The mitochondrial form is generated by the removal of an N-terminal extension that targets the enzyme to the mitochondrion .
FH is essential for cellular metabolism and energy production. It facilitates a transition step in the production of energy in the form of NADH . In the cytosol, FH is involved in the urea cycle and amino acid metabolism, where it plays a role in the dehydration of L-malate to fumarate . Additionally, FH has been implicated in DNA repair by promoting non-homologous end-joining (NHEJ) .
Mutations in the FH gene can lead to fumarase deficiency, a rare metabolic disorder that causes severe neurological impairment and progressive encephalopathy . FH mutations are also associated with hereditary leiomyomatosis and renal cell cancer (HLRCC), a condition characterized by the development of benign smooth muscle tumors and an increased risk of renal cell carcinoma .
Mouse anti-human fumarate hydratase antibodies are monoclonal antibodies produced by immunizing mice with human fumarate hydratase protein. These antibodies are used in various research applications to detect and study the FH protein in human tissues and cells .
Monoclonal antibodies are produced by hybridoma technology, which involves the fusion of mouse myeloma cells with spleen cells from immunized mice. The resulting hybrid cells, or hybridomas, are capable of producing large quantities of specific antibodies . Mouse anti-human FH antibodies are typically of the IgG class and can be used in techniques such as Western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry (IHC), and enzyme-linked immunosorbent assay (ELISA) .
These antibodies are valuable tools for studying the expression, localization, and function of FH in various biological contexts. They can help researchers investigate the role of FH in metabolic pathways, its involvement in disease processes, and its potential as a therapeutic target .
