Schematic representation of fumarate hydratase (FH) protein, indicating its functional Pfam (protein families) Database (European Molecular Biology Laboratory, Heidelberg, Germany) domains lyase (green) and fumarase
(FUM) converts fumarate into malate, which is oxidized by malate dehydrogenase (MDH) again producing one NADH and regenerating the initial oxalacetate so the cycle can start over again.
deficiency is an autosomal recessive encephalopathy affecting both the mitochondrial and the cytosolic enzymes.
vannamei FE123204.1 - Fumarase
No record - - 2-Oxoglutarate dehydrogenase L.
Beneficial elements are used to increase SMs; for example, the content of a-tocopherol, asparagine and tyrosine (Hediji et al., 2010), isocitrate dehydrogenase (ICDH), citrate synthase (CS), fumarase
, malate dehydrogenase (MDH) and phosphoenolpyruvate carboxylase (PEPC) increases in tomato plants (Lopez-Millan et al., 2009) when 100 uM Cd is used in the NS.
If a patient is admitted with a suspected kidney injury, we can screen the injury using blood/urine with the help of an enzyme called fumarase
, and then a scan can pinpoint where in the tissue the injury is.
It has also been suggested that necrosis can be detected using hyperpolarized [sup.13]C-fumarate and its extra-cellular conversion to [sup.13]C-malate via fumarase
, an enzyme that presents in cytosol and mitochondria .
Growth and development failure, hypotonia, seizures and brain atrophy are the common characteristics of patients with fumarase
Shannon Mason, 11, has Fumarase
Deficiency - which causes frequent seizures, limited speech and means she is partially sighted.
The possibility of complicated curves for simple kinetic schemes and the computer fitting of experimental data for acetylcholinesterase, acid phosphatase, adenosine deaminase, arylsulphatase, benzylamine oxidase, chymotrypsin, fumarase
, galactose dehydrogenase, [beta]-galactosidase, lactate dehydrogenase, peroxidase and xanthine oxidase", Biochemical Journal 1980; 187: 739-765.
26s protease subunit 2.34 (Sus scrofa) 8633 P10173 Fumarate hydratase, mitochondrial 2.32 (Fumarase
) 6226 P00939 Triosephosphate isomerase (TIM) 1.45 Expression of pregnancy phase 2509 P48616 Vimentin 2.37 4223 XP_218574 Similar to BC013491 protein 1.03 (Rattus norvegicus) 5212 P13620 ATP synthase D chain, 2.00 mitochondrial 6311 1VSEB Chain B, Crystal structure 2.30 analysis of bovine carbonic anhydrase II 6224 P41976 Superoxide dismutase (Mn), 2.38 Mitochondrial precursor 7813 AAC60522 Manganous superoxide dismutase; 2.37 MnSOD (Bos taurus) * AC = Accession Code (NCBI search).
Katlyn has a rare neurological disease called fumarase
deficiency that severely limits physical movement and can lead to death.