• 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). SDH1 and SDH2 form the catalytic dimer. (ymdb.ca)
  • Cloning and characterization of the iron-sulfur subunit gene of succinate dehydrogenase from Saccharomyces cerevisiae. (ymdb.ca)
  • Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Succinate Dehydrogenase Complex Subunit D (SDHD) were tested 20 times on one plate, respectively. (biomatik.com)
  • Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Succinate Dehydrogenase Complex Subunit D (SDHD) were tested on 3 different plates, 8 replicates in each plate. (biomatik.com)
  • This assay has high sensitivity and excellent specificity for detection of Succinate Dehydrogenase Complex Subunit D (SDHD). (biomatik.com)
  • No significant cross-reactivity or interference between Succinate Dehydrogenase Complex Subunit D (SDHD) and analogues was observed. (biomatik.com)
  • Wang JH, Lasota J, Miettinen M.: Succinate Dehydrogenase Subunit B (SDHB) Is Expressed in Neurofibromatosis 1-Associated Gastrointestinal Stromal Tumors (Gists): Implications for the SDHB Expression Based Classification of Gists. (biopticka.cz)
  • Renal tumours have recently been described in association with mutations in the gene encoding the B subunit of succinate dehydrogenase, a mitochondrial Krebs cycle and electron transport chain enzyme (SDHB-associated renal cell carcinomas). (dundee.ac.uk)
  • Succinate dehydrogenase-deficient RCC is diagnosed by immunohistochemical (IHC) staining for SDH subunit B (SDHB), with loss of staining confirming SDHB deficiency. (sajo.org.za)
  • In step 6 of the citric acid cycle, SQR catalyzes the oxidation of succinate to fumarate with the reduction of ubiquinone to ubiquinol. (wikipedia.org)
  • Pheocromocytoma-Paraganglioma syndromes are associated with mutations in 3 different genes that encode subunits B, C, and D of the mitochondrial enzyme succinate dehydrogenase (SDH), which catalyzes the oxidation of succinate to fumarate in the Krebs cycle [1-2]. (snmjournals.org)
  • SDH transports electrons to the ubiquinone (coenzyme Q) pool in the respiratory chain and catalyzes the oxidation of succinate to fumarate in the TCA cycle. (scientificarchives.com)
  • SDHA is the primary catalytic component that changes succinate into fumarate. (scientificarchives.com)
  • We will explore the independent roles of elevated SDHA, SDHA substrates (succinate, fumarate), or MPC1 in reprograming of cellular metabolism and cell proliferation. (grantome.com)
  • Subphenotypic LRs exceeded 6 for invasive disease (SDHB), head -and- neck disease (SDHD), multiple tumors (SDHD), family history of PCC/PGL, loss of SDHB staining on immunohistochemistry , and succinate -to- fumarate ratio >97 (SDHB, SDHD). (bvsalud.org)
  • Complex II catalyzes the oxidation of succinate to fumarate (Succinate dehydrogenase: sdh) and transfers i. (bvsalud.org)
  • The enzyme has a central role in carbon and energy metabolism by catalyzing the oxidation of succinate to form fumarate coupled to the reduction of quinone in the respiratory chain. (lu.se)
  • Clinical and molecular genetics of patients with the Carney-Stratakis syndrome and germline mutations of the genes coding for the succinate dehydrogenase subunits SDHB, SDHC, and SDHD. (biopticka.cz)
  • 2 , 4 , 5 Succinate dehydrogenase consists of four subunits, namely subunits A-D (SDHA, SDHB, SDHC and SDHD), all encoded by autosomal deoxyribonucleic acid (DNA). (sajo.org.za)
  • Aberrant Splicing of SDHC in Families With Unexplained Succinate Dehydrogenase-Deficient Paragangliomas. (cdc.gov)
  • Quantifying evidence toward pathogenicity for rare phenotypes: The case of succinate dehydrogenase genes, SDHB and SDHD. (bvsalud.org)
  • SDH mutation caused enzymatic activity change in the mitochondrial electron transport chain can activate a hypoxic-angiogenic pathway with an accumulation of succinate and the transcription of a number of genes involved in tumorigenesis, angiogenesis, and resistance to apoptosis [1-2]. (snmjournals.org)
  • succinate dehydrogenase, Sdh) genes cause familiar pheochromocytoma/paraganglioma tumors. (ed.ac.uk)
  • Familial pheochromocytomas and carotid body tumors may be due to mutations in genes encoding the enzyme succinate dehydrogenase or other signaling molecules. (msdmanuals.com)
  • Succinate dehydrogenase (SDH) or succinate-coenzyme Q reductase (SQR) or respiratory complex II is an enzyme complex, found in many bacterial cells and in the inner mitochondrial membrane of eukaryotes. (wikipedia.org)
  • SQR consists of a membrane-peripheral heterodimer domain (often called succinate dehydrogenase) which is tightly bound to a membrane-spanning anchor, which is a cytochrome with one or two heme groups depending on the organism. (lu.se)
  • From a comprehensive analysis of metabolic pathways in our patient-derived ovarian tumors and The Cancer Genome Atlas (TCGA) data, we discovered that a key mitochondrial enzyme, succinate dehydrogenase (SDHA), is significantly upregulated in 19% of ovarian cancer patients, and is associated with significantly improved patient survival. (grantome.com)
  • SdhA provides the binding site for the oxidation of succinate. (wikipedia.org)
  • The succinate-binding site and ubiquinone-binding site are connected by a chain of redox centers including FAD and the iron-sulfur clusters. (wikipedia.org)
  • Succinate dehydrogenase assembly factor 2 (SDHAF2), also encoded by autosomal DNA, combines the subunits at the inner mitochondrial membrane where it forms mitochondrial complex 2 (SDH/succinate-ubiquinone oxireductase). (sajo.org.za)
  • Complex II (Succinate-ubiquinone oxidoreductase) is an importnat enzyme complex for the tricarboxylic acid cycle and the aerobic respiratory chain of motochondria and procaryotic organisms (12, 24). (bvsalud.org)
  • Fig. 2: Succinate controls mitochondrial superoxide production through mitochondrial complex I. (nature.com)
  • Succinate dehydrogenase (SDH) is an enzyme complex composed of four major subunits (A,B, C and D). It is localized on the inner side of the mitochondrial membrane and is part of the citric acid cycle and respiratory chain. (biopticka.cz)
  • Succinate dehydrogenase (SDH)-deficient renal carcinoma: a morphologically distinct entity: a clinicopathologic series of 36 tumors from 27 patients. (librepathology.org)
  • Succinate dehydrogenase-deficient renal cell carcinoma: detailed characterization of 11 tumors defining a unique subtype of renal cell carcinoma. (librepathology.org)
  • Learning Objectives Succinate dehydrogenase deficiency (type B) is associated with Pheochromocytoma-Paraganglioma syndrome, which can result in wide metastatic extra-adrenal paragangliomas. (snmjournals.org)
  • The patient was identified with succinate dehydrogenase deficiency (type B), which is associated with Pheochromocytoma-Paraganglioma syndrome, Type 4, and can result in extra-adrenal paragangliomas which tend to metastasize widely. (snmjournals.org)
  • Succinate:quinone oxidoreductase (SQR) is both a Krebs´ citric acid cycle enzyme and a component (Complex II) of the respiratory chain in aerobic cells. (lu.se)
  • Histochemical analysis showing high succinate dehydrogenase in muscle demonstrates high mitochondrial content and high oxidative potential. (wikipedia.org)
  • An enzyme that plays a role in the GLUTAMATE and butanoate metabolism pathways by catalyzing the oxidation of succinate semialdehyde to SUCCINATE using NAD+ as a coenzyme. (harvard.edu)
  • When oxygen is limited, as in muscles that have undergone prolonged exercise, pyruvate is not consumed in the TCA cycle but is rather converted into lactic acid by lactate dehydrogenase (LDH) in a process termed anaerobic glycolysis. (hindawi.com)
  • Such enzymes include the pyruvate dehydrogenase complex that synthesises the acetyl-CoA needed for the first reaction of the TCA cycle. (citizendium.org)
  • This includes both the entry point enzymes pyruvate dehydrogenase and citrate synthase. (citizendium.org)
  • In the presence of oxygen, the pyruvate is directed into mitochondria to be converted into acetyl CoA by the pyruvate dehydrogenase (PDH) or into alanine by transamination. (hindawi.com)
  • Establishment and validation of a clinical severity scoring system for succinic semialdehyde dehydrogenase deficiency. (harvard.edu)
  • Understanding the Molecular Mechanisms of Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD): Towards the Development of SSADH-Targeted Medicine. (harvard.edu)
  • Development of a Quality-of-Life Survey for Patients With Succinic Semialdehyde Dehydrogenase Deficiency, a Rare Disorder of GABA Metabolism. (harvard.edu)
  • Transcranial Magnetic Stimulation in Succinic Semialdehyde Dehydrogenase Deficiency: A Measure of Maturational Trajectory of Cortical Excitability. (harvard.edu)
  • aminobutyric acid B (GABA-B) Receptor Antagonist, for Succinic Semialdehyde Dehydrogenase Deficiency. (harvard.edu)
  • Speech Motor Function and Auditory Perception in Succinic Semialdehyde Dehydrogenase Deficiency: Toward Pre-Supplementary Motor Area (SMA) and SMA-Proper Dysfunctions. (harvard.edu)
  • Postmortem Analyses in a Patient With Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD): II. (harvard.edu)
  • Magnetic Resonance Imaging (MRI) and Spectroscopy in Succinic Semialdehyde Dehydrogenase Deficiency. (harvard.edu)
  • Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare inborn error of metabolism that is inherited in an autosomal recessive pattern. (rarediseases.org)
  • Among these, the citric acid cycle intermediate succinate stands out owing to its multiple roles in disparate pathways, its dramatic concentration changes and its selective cell release. (nature.com)
  • Use of the DNA polymerase chain reaction for homology probing: isolation of partial cDNA or genomic clones encoding the iron-sulfur protein of succinate dehydrogenase from several species. (ymdb.ca)
  • Here we propose that succinate has evolved as a signaling modality because its concentration reflects the coenzyme Q (CoQ) pool redox state, a central redox couple confined to the mitochondrial inner membrane. (nature.com)
  • Succinate dehydrogenase deficiency contributes to the predisposition to metastatic paragangliomas. (snmjournals.org)
  • Succinate-Semialdehyde Dehydrogenase" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)
  • This graph shows the total number of publications written about "Succinate-Semialdehyde Dehydrogenase" by people in Harvard Catalyst Profiles by year, and whether "Succinate-Semialdehyde Dehydrogenase" was a major or minor topic of these publication. (harvard.edu)
  • Below are the most recent publications written about "Succinate-Semialdehyde Dehydrogenase" by people in Profiles. (harvard.edu)
  • Catalyzes the NADPH-dependent reduction of succinyl-CoA to succinate semialdehyde. (expasy.org)
  • The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. (biopticka.cz)
  • We also present a thorough analysis of the role of succinate in the initiation of metabolic and epigenetic changes that contribute to the development of cancer, as well as its effects on angiogenesis, cell invasion, and migration. (scientificarchives.com)
  • In this review, we talk about new research on changes to SDH activity, such as SDH dysregulation or endogenous SDH inhibitors that cause succinate buildup. (scientificarchives.com)
  • Succinate dehydrogenase is a respiratory enzyme that forms part of both the respiratory chain and the tricarboxylic acid pathway, or Krebs cycle. (sajo.org.za)
  • Murphy, M. P. & O'Neill, L. A. J. Krebs cycle reimagined: the emerging roles of succinate and itaconate as signal transducers. (nature.com)
  • Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancer. (librepathology.org)
  • However, the E. coli cells did not show increased succinate dehydrogenase activity nor did the operon complement a sdhCDAB defective E. coli mutant [7]. (lu.se)
  • For instance, a complicated network of interactions connects a cell's bioenergetic features and neoplastic potential through the imbalance of sirtuin 3 (SIRT3) and succinate dehydrogenase (SDH) enzymatic activity in mitochondria. (scientificarchives.com)
  • Succinate dehydrogenase (SDH) activity in hair cells: a correlate for permanent threshold elevations. (cdc.gov)
  • The logic of this form of regulation explains many emerging roles of succinate in biology, and suggests future research questions. (nature.com)
  • Fig. 3: Accumulated mitochondrial succinate regulates cellular αKG-dependent dioxygenases. (nature.com)
  • The aim of the study was to assess the indications for succinate dehydrogenase (SDH) genetic testing in a prospective study. (qxmd.com)
  • Mitochondrial succinate dehydrogenase function is essential for sperm motility and male fertility. (urotoday.com)