SDHB Recombinant Rabbit Monoclonal Antibody [JU02-33]
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 associate 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.
Product Specifications
CAS Number
9000-83-3
Product Name Alternative
Abbreviation
Ip, SDHB, SDH, SDH1
Swiss Prot
P21912 Human, Q9CQA3 Mouse, P21913 Rat
Cellular Locus
Mitochondrion. Mitochondrion inner membrane.
Host
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Synthetic peptide within Human SDHB aa 201-250 / 280.
Isotype
IgG
Conjugation
Non-conjugated
Type
Recombinant Rabbit monoclonal Antibody
Applications
WB, IHC-P
Positive Control
Concentration
1 mg/mL
Dilution
WB: 1:1,000-1:2,000 ;IHC-P: 1:1,000
Purity
Protein A affinity purified.
Form
Liquid
Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Molecular Weight
Predicted band size: 32 kDa
Storage Conditions
Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Recombinant Antibody
Yes
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