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SDHD 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). 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). 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. Belongs to the CybS family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Energy Metabolism - oxidative phosphorylation; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial
Chromosomal Location of Human Ortholog: 11q23.1
Cellular Component: integral component of membrane; mitochondrial envelope; mitochondrial inner membrane; mitochondrial respiratory chain complex II; mitochondrion
Molecular Function: electron transfer activity; heme binding; metal ion binding; succinate dehydrogenase (ubiquinone) activity; succinate dehydrogenase activity; ubiquinone binding
Biological Process: mitochondrial electron transport, succinate to ubiquinone; tricarboxylic acid cycle
Disease: Carcinoid Tumors, Intestinal; Cowden Syndrome 3; Mitochondrial Complex Ii Deficiency; Paraganglioma And Gastric Stromal Sarcoma; Paragangliomas 1; Pheochromocytoma
Reference #:  O14521 (UniProtKB)
Alt. Names/Synonyms: CBT1; CII-4; CybS; DHSD; PGL; PGL1; QPs3; SDH4; SDHD; Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Succinate dehydrogenase complex subunit D; succinate dehydrogenase complex, subunit D, integral membrane protein; succinate dehydrogenase ubiquinone cytochrome B small subunit; Succinate-ubiquinone oxidoreductase cytochrome b small subunit; Succinate-ubiquinone reductase membrane anchor subunit
Gene Symbols: SDHD
Molecular weight: 17,043 Da
Basal Isoelectric point: 8.92  Predict pI for various phosphorylation states
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