| Weight | 1 lbs |
|---|---|
| Dimensions | 9 × 5 × 2 in |
| host | mouse |
| isotype | IgG |
| clonality | monoclonal |
| concentration | concentrate, predilute |
| applications | IHC |
| reactivity | human |
| available size | 0.1 mL, 0.5 mL, 1 mL concentrated, 7 mL prediluted |
rabbit anti-SDHB monoclonal antibody (ZR339) 6362
Price range: $160.00 through $528.00
Antibody summary
- Rabbit monoclonal to SDHB
- Suitable for: Immunohistochemistry (formalin-fixed, paraffin-embedded tissues)
- Reacts with: Human
- Isotype:IgG
- Control: Paraganglioma
- Visualization: Cytoplasmic
- 0.1, 0.5, 1.0 mL concentrated, 7 mL prediluted
rabbit anti-SDHB monoclonal antibody ZR339 6362
| target relevance |
|---|
| Homo sapiens SDHB Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial |
| Protein names Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial |
| Alternative names Iron-sulfur subunit of complex II, Malate dehydrogenase [quinone] iron-sulfur subunit |
| Gene names SDHB |
| Protein family Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family |
| Function Iron-sulfur protein (IP) subunit of the succinate dehydrogenase complex (mitochondrial respiratory chain complex II), responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:26925370, PubMed:27604842). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity) |
| Catalytic activity a quinone + succinate = fumarate + a quinol (R)-malate + a quinone = enol-oxaloacetate + a quinol (S)-malate + a quinone = enol-oxaloacetate + a quinol |
| Subcellular location Mitochondrion inner membrane |
| Structure (Microbial infection) Interacts with JC virus small t antigen |
| Involvement in disease Pheochromocytoma/paraganglioma syndrome 4 A form of pheochromocytoma/paraganglioma syndrome, a tumor predisposition syndrome characterized by the development of neuroendocrine tumors, usually in adulthood. Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells in the adrenal medulla. Paragangliomas develop from sympathetic paraganglia in the thorax, abdomen, and pelvis, as well as from parasympathetic paraganglia in the head and neck. PPGL4 inheritance is autosomal dominant. Paraganglioma and gastric stromal sarcoma Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance. Mitochondrial complex II deficiency, nuclear type 4 A form of mitochondrial complex II deficiency, a disorder with heterogeneous clinical manifestations. Some patients have multisystem involvement of the brain, heart, muscle, liver, and kidneys resulting in death in infancy, whereas others have only isolated cardiac or muscle involvement with onset in adulthood and normal cognition. 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. MC2DN4 is a severe, autosomal recessive form characterized by early-onset progressive neurodegeneration with leukoencephalopathy. |
| Keywords 2Fe-2S, 3D-structure, 3Fe-4S, 4Fe-4S, Acetylation, Disease variant, Electron transport, Host-virus interaction, Iron, Iron-sulfur, Membrane, Metal-binding, Mitochondrion, Mitochondrion inner membrane, Oxidoreductase, Primary mitochondrial disease, Proteomics identification, Reference proteome, Transit peptide, Transport, Tricarboxylic acid cycle |
| Sequence MAAVVALSLRRRLPATTLGGACLQASRGAQTAAATAPRIKKFAIYRWDPDKAGDKPHMQT YEVDLNKCGPMVLDALIKIKNEVDSTLTFRRSCREGICGSCAMNINGGNTLACTRRIDTN LNKVSKIYPLPHMYVIKDLVPDLSNFYAQYKSIEPYLKKKDESQEGKQQYLQSIEEREKL DGLYECILCACCSTSCPSYWWNGDKYLGPAVLMQAYRWMIDSRDDFTEERLAKLQDPFSL YRCHTIMNCTRTCPKGLNPGKAIAEIKKMMATYKEKKASV |
| UniProt accession: P21912 |
Data
 |
| Formalin-fixed, paraffin-embedded succinate dehydrogenase deficient RCC stained with anti-SDHB antibody using peroxidase-conjugate and DAB chromogen. Note the cytoplasmic staining of tumor cells |
FAQ & Publications
Frequently Asked Questions
What species is the rabbit anti-SDHB monoclonal antibody (ZR339) reactive with?
This antibody is reactive with human species.
What are the recommended storage conditions for the rabbit anti-SDHB monoclonal antibody?
For short-term storage, keep the antibody at 2-8°C. For long-term storage, store it at -20°C and avoid freeze/thaw cycles.
Which applications is the rabbit anti-SDHB monoclonal antibody validated for, and what are the suggested dilutions?
The antibody is validated for immunohistochemistry (IHC) on formalin-fixed, paraffin-embedded tissues. The recommended dilution for the concentrated antibody is 1:100-200.
Publications
| pmid | title | authors | citation |
|---|---|---|---|
| We haven't added any publications to our database yet. | |||
Published literature highly relevant to the biological target of this product and referencing this antibody or clone are retrieved from the PubMed database provided by the United States National Library of Medicine at the National Institutes of Health.
Protocols
| relevant to this product |
|---|
| IHC |
Documents
| Batch Number | QC File | SDS |
|---|---|---|
| To view batch-specific Safety Datasheets and Quality Certificates associated with your account, please Log In. | ||
Only logged in customers who have purchased this product may leave a review.
Reviews
There are no reviews yet.