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Antibody Type: Rabbit Polyclonal
Application: IHC-P,IHC-Fr,IF,FCM
Reactivity: Human,Mouse (predicted:Rat)

| Description | Anti-SDHA Polyclonal Antibody is a Rabbit antibody targeting SDHA. Anti-SDHA Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF,FCM. |
| Ig Type | IgG |
| Reactivity | Human,Mouse (predicted:Rat) |
| Application | |
| Recommended Dose | IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500; FCM: 2μg/Test |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Construction | Hybridoma Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research 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. |
| Immunogen | KLH conjugated synthetic peptide: human SDHA |
| Antigen Species | Human |
| Gene Name | SDHA |
| Gene ID | |
| Protein Name | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial |
| Uniprot ID | |
| Function | Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D). A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. |
| Molecular Weight | Theoretical: 70 kDa. |
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
| Size | Quantity | Unit Price | Amount | Operation |
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