Clinical manifestations of familial paraganglioma and phaeochromocytomas in succinate dehydrogenase
B (SDH-B) gene mutation carriers.
Hereditary paraganglioma/ pheochromocytoma and inherited succinate dehydrogenase
Korpershoek et al., "Succinate dehydrogenase
(SDH) deficient pancreatic neuroendocrine tumor expands the SDH-related tumor spectrum," The Journal of Clinical Endocrinology & Metabolism, vol.
Gene mutations in the succinate dehydrogenase
subunitSDHBcause susceptibility to familial pheochromocytoma and to familial paraganglioma.
activity: Purple formazan deposit was observed in MII oocytes with succinate dehydrogenase
reaction (Figure 1).
is involved in both citric acid cycle and electron transport chain and plays essential roles in oxidative metabolism together with PGC-1[alpha].
Phenazine oxidoreductase (soluble succinate dehydrogenase
- SDH) was measured in terms of the decrease in absorbance due to the reduction in 2,6-dichloroindophenol (DCIP) at 600 nm as reference wavelength ([epsilon] = 19.1 [mM.sup.-1] x [cm.sup.-1]) in the presence of phenazine methosulphate (PMS).
was assayed according to the method of Slater and Bonner (1952) The succinate dehydrogenase
activity was expressed as nmol of succinate oxidised/min/mg protein.
As many as 30% of paragangliomas can be caused by inheritance of mutations in protein subunits of succinate dehydrogenase
, a critical enzyme within complex II of the mitochondrial respiratory chain.
Histochemical reactions were estimated to elucidate activity of succinate dehydrogenase
, EC 22.214.171.124, (SDH) and L-lactate dehydrogenase, EC 126.96.36.199, (LDH) .
At the cellular level, 3NPA inhibits succinate dehydrogenase
[4, 5], a key enzyme of oxidative energy production that is localized in the mitochondrial inner membrane and complex II of the respiratory chain, causing ATP levels in the brain to fall.
New-population females were more often missing a small part of the succinate dehydrogenase
gene (Sdhd) and this small deletion was associated with the ability to maintain flight for a greater duration.