Trial Size Available
Carrier-Free Available
| Document Name | |
|---|---|
| Datasheet | Download Here |
| Material Safety Data Sheet | Download Here |
| Applications | Tested Dilutions | Protocol | Note |
|---|---|---|---|
| Immunohistochemistry (IHC) | 1-2ug/ml | IHC Protocol | 30 min at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes |
In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol. Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB) and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane. Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders. Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations are associated with juvenile encephalopathy as well as Leigh syndrome, a severe neurological disorder. Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma.
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