1-832-868-1888
order@swbio.com

Catalog Number: |
49524 |
| other_names: | CSNBAD1 antibody MGC138309 antibody MGC138311 antibody OPN 2 antibody OPN2 antibody opsd antibody OPSD_HUMAN antibody opsin 2 antibody Opsin 2 rod pigment antibody Opsin-2 antibody Opsi |
Amount: |
100μg |
| calculated_mw: | 39 kDa |
| host_species: | Rabbit |
Price: |
$319 |
Swiss-Prot No: |
Swiss-Prot#:P08100 |
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, IHC |
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: |
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appl_detail: |
WB: 1:500-1:1,000 |
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