SDHA Antibody

Catalog Number:

49453

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P31040

Form of Antibody:

1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.

Storage/Stability:

Immunogen:

recombinant protein

Purification:

ProA affinity purified

Specificity/Sensitivity:

Applications:

WB, ICC/IF, IHC, FC

Background:

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.

References:

appl_detail:

WB: 1:500-1:1,000
IHC: 1:50-1:200
ICC: 1:50-1:200

FC: 1:50-1:100

Datasheet PDF