fumarate

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Related to Fumarates: fumaric acid esters

fumarate

 [fu´mar-āt]
a salt of fumaric acid.

fumarate

/fu·ma·rate/ (fu´mah-rāt) a salt of fumaric acid.

fumarate hydratase

An enzyme encoded by FH on chromosome 1q42.1, which catalyses the reversible hydration of fumarate to S-malate. The mitochondrial isoform is involved in the Krebs (citric acid) cycle; the cytosol isoform is involved in amino-acid-reductive (carboxylation cycle—CO2 fixation) metabolism.

Abnormal expression
FH mutations can cause fumarase deficiency and lead to progressive encephalopathy, see table.

Molecular defects
Fumarase deficiency in fetus
Polyhydramnios, brain defects.

Fumarase deficiency in neonate
Severe neurologic defects, poor feeding, failure to thrive, and hypotonia; attributed to defects in the mitochondrial and cytosol isoforms.

Fumarate hydratase germ-line mutations trigger an array of tumours: Uterine leiomyomatosis and renal-cell carcinoma (Reed’s syndrome).

fumarate

a salt of fumaric acid.
References in periodicals archive ?
These studies reported similar outcomes to the randomized clinical trials: significant reductions in the extent and severity of psoriasis with fumarate treatment, and frequent adverse effects, predominantly GI problems, flushing, and laboratory abnormalities.
The running hypothesis is that dimethyl fumarate works by correcting the immunologic imbalance in psoriasis (shifting from a Th1/TH17 pattern of immune response to a Th2 one).
M2 EQUITYBITES-January 7, 2014-Alkermes plc announces USPTO's Notice of Allowance for novel fumarate prodrug for multiple sclerosis
The company added that the allowed composition of matter claims will cover its MMF prodrug, ALKS 8700, a proprietary, small-molecule prodrug of monomethyl fumarate (MMF) for the treatment of multiple sclerosis.