|Immunocytochemistry (ICC)||5 ug/ml|
|Immunohistochemistry (IHC)||Assay Dependent|
|Immunoprecipitation (IP)||Assay Dependent|
|Tested Species reactivity||Bovine, Human, Rat|
|Host / Isotype||Mouse / IgG2a|
|Immunogen||Purified proteins from bovine liver, bovine heart, porcine heart.|
|Contains||0.02% sodium azide|
|Storage conditions||4° C|
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: Fumarase; fumarate hydratase; fumarate hydratase 1; Fumarate hydratase, mitochondrial; HLRCC; LRCC; MCL; MCUL1; mitochondrial
Gene Aliases: FH; Fh1; FMRD; HLRCC; LRCC; MCL; MCUL1
UniProt ID: (Human) P07954
Molecular Function: lyase
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