fumarate

fu·ma·rate

F0360100 (fyo͞o′mə-rāt′)n. A salt or ester of fumaric acid.

[fumar(ic acid) + -ate.]

fumarate

(ˈfjuːməˌreɪt) n (Biochemistry) biochem a salt of fumaric acid produced during the urea cycle

fu•ma•rate

(ˈfyu məˌreɪt)

n. the salt of fumaric acid, a key chemical intermediate in the Krebs cycle. [1860–65]

fumarate

enUK

fumarate

[fu´mar-āt] 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).

单词	fumarate
释义	fumarate enUK fu·ma·rate F0360100 (fyo͞o′mə-rāt′)n. A salt or ester of fumaric acid. [fumar(ic acid) + -ate.] fumarate (ˈfjuːməˌreɪt) n (Biochemistry) biochem a salt of fumaric acid produced during the urea cycle fu•ma•rate (ˈfyu məˌreɪt) n. the salt of fumaric acid, a key chemical intermediate in the Krebs cycle. [1860–65] fumarate enUK fumarate [fu´mar-āt] 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).
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