The protein encoded by this gene is an enzymatic component
of the tricarboxylic acid (TCA) 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.
