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a salt of fumaric acid.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

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).
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
References in periodicals archive ?
(Nasdaq: BIIB) today announced positive topline results from EVOLVE-MS-2, a large, randomized, double-blind, five-week, Phase 3 study of diroximel fumarate, an investigational, novel oral fumarate with a distinct chemical structure, for relapsing-remitting multiple sclerosis (RRMS), compared to TECFIDERA[R] (dimethyl fumarate).
Diroximel fumarate was statistically superior to dimethyl fumarate on the study's pre-specified primary endpoint, with patients treated with diroximel fumarate self-reporting significantly fewer days of key gastrointestinal (GI) symptoms with intensity scores 2 on the Individual Gastrointestinal Symptom and Impact Scale (IGISIS), as compared to dimethyl fumarate (p=0.0003).
[ClickPress, Tue Jul 09 2019] The diisostearyl fumarate market is primarily driven by the rising adoption of cosmetic products by millennial and trends in skin care, hair care, and cosmetics globally.
The approval was based on positive results from the Phase III KRONOS trial in which Breztri Aerosphere demonstrated a statistically-significant improvement in trough forced expiratory volume in one second (FEV1), the primary endpoint for Japan, compared with the dual comparators Bevespi Aerosphere (glycopyrronium/formoterol fumarate) and PT009 (budesonide/formoterol fumarate).
In this study, standard samples of ferrous fumarate and gluconate were measured using Mossbauer spectroscopy.
First introduced in the 1950s because it was thought that psoriasis was caused by a biochemical defect of the citric acid (Krebs) cycle, evidence suggests that it has nothing to do with the Krebs cycle, and the major active compound appears to be dimethyl fumarate. 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).
Compared to formulations containing tenofovir disoproxil fumarate, the newer tenofovir alafenamide formulation achieves higher intracellular levels in CD4 lymphocytes (but not in renal tubular cells).
The FDA also has granted the long-acting beta-2 agonist formoterol fumarate tentative approval for use as monotherapy.
She first heard about dimethyl fumarate at an outpatient appointment at the RVI, where her consultant mentioned that the treatment was being tested.
Researchers found that the periodontal biofilm depended for growth on the availability of iron and heme (an iron-containing molecule related to hemoglobin), and that restricting these reduced levels of the enzyme, fumarate reductase.
In March, oral Tecfidera[TM] (dimethyl fumarate, Biogen Idec--formerly "BG-12") joined the ranks of first-line, disease-modifying therapies approved by the U.S.
Ketotifen fumarate, chemically known as 4-(1-methyl-4piperidylidene)-4h-benzo [4,5] cyclohepta [1,2-b] thiophen-10(9h)-one hydrogen fumarate (Figure 2), is cycloheptathiophene blocker of histamine h-1 receptors used as an antiallergic and an antiasthmatic drug [9].