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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H, M | WB, ICC | Rb | Affinity Purified | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | ABT110 |
| Description | Anti-SDHD (CybS) Antibody |
| Alternate Names |
|
| Background Information | Succinate dehydrogenase complex subunit D (SDHD) is a member of the CybS family and is one of four subunits of the succinate dehydrogenase (SDH) complex involved in the electron transport chain of the mitochondria. The specific role of SDH is to transfer electrons from succinate to ubiquinone, and the SDHD subunit is involved in anchoring the SDH complex to the inner mitochondrial membrane. Defective SDHD has been linked to Carney-Stratakis syndrome, a condition characterized by concurrent paraganglioma and gastric stromal sarcomas. |
| Product Information | |
|---|---|
| Format | Affinity Purified |
| Control |
|
| Presentation | Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | This Anti-SDHD (CybS) Antibody is validated for use in Western Blotting, ICC for the detection of SDHD (CybS). |
| Key Applications |
|
| Application Notes | Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected SDHD (CybS) in NIH/3T3 and HeLa cells. |
| Biological Information | |
|---|---|
| Immunogen | KLH-conjugated linear peptide corresponding to the Topological domain of human SDHD (CybS). |
| Epitope | Topological domain (mitochondrial matrix) |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Rabbit |
| Specificity | This antibody recognizes the Topological domain (mitochondrial matrix) of SDHD (CybS). |
| Species Reactivity |
|
| Species Reactivity Note | Demonstrated to react with Human and Mouse. Predicted to react with Bovine, Sheep, and Rhesus Macaque based on 100% sequence homology. Other homologies: Rat (92% sequence homology). |
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | Complex II of the respiratory chain, which is specifically involved in the oxidation of succinate, carries electrons from FADH to CoQ. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. The subunit D protein is one of two integral membrane proteins anchoring the complex to the matrix side of the membrane. Mutations in SDHD have been linked to hereditary paraganglioma. |
| Gene Symbol |
|
| Purification Method | Affinity Purfied |
| UniProt Number |
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| UniProt Summary | FUNCTION: Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle. SUBUNIT STRUCTURE: Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD. SUBCELLULAR LOCATION: Mitochondrion inner membrane; Multi-pass membrane protein. INVOLVEMENT IN DISEASE: Defects in SDHD are a cause of paragangliomas type 1 (PGL1). A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. PGL1 is a rare autosomal dominant disorder which is characterized by the development of mostly benign, highly vascular, slowly growing tumors in the head and neck. In the head and neck region, the carotid body is the largest of all paraganglia and is also the most common site of the tumors. Defects in SDHD are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. Defects in SDHD may be a cause of susceptibility to intestinal carcinoid tumor (ICT). A yellow, well-differentiated, circumscribed tumor that arises from enterochromaffin cells in the small intestine or, less frequently, in other parts of the gastrointestinal tract. Defects in SDHD are a cause of paraganglioma and gastric stromal sarcoma (PGGSS); also called Carney-Stratakis syndrome. 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. Defects in SDHD are a cause of Cowden-like syndrome (CWDLS). Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus. SEQUENCE SIMILARITIES: Belongs to the CybS family. |
| Molecular Weight | ~11 kDa observed. An uncharacterized band may be observed at ~25 kDa in some cell lysates. |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blot in HeLa cell lysate. Western Blot Analysis: 1 µg/mL of this antibody detected SDHD (CybS) in 10 µg of HeLa cell lysate. |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |