scholarly journals Succinate dehydrogenase assembly factor 2 is needed for assembly and activity of mitochondrial complex II and for normal root elongation in Arabidopsis

2012 ◽  
Vol 73 (3) ◽  
pp. 429-441 ◽  
Author(s):  
Shaobai Huang ◽  
Nicolas L. Taylor ◽  
Elke Ströher ◽  
Ricarda Fenske ◽  
A. Harvey Millar
Keyword(s):  
Succinate Dehydrogenase ◽  
Root Elongation ◽  
Mitochondrial Complex ◽  
Complex Ii ◽  
Assembly Factor

scholarly journals Succinate dehydrogenase (SDHx) mutations in pituitary tumors: could this be a new role for mitochondrial complex II and/or Krebs cycle defects?

2012 ◽  
Vol 19 (6) ◽  
pp. C33-C40 ◽  
Author(s):  
Paraskevi Xekouki ◽  
Constantine A Stratakis
Keyword(s):  
Thyroid Cancer ◽  
Succinate Dehydrogenase ◽  
Krebs Cycle ◽  
Pituitary Tumors ◽  
Tricarboxylic Acid ◽  
Genetic Defects ◽  
Disease Phenotype ◽  
Cowden Disease ◽  
Mitochondrial Complex ◽  
Complex Ii

Succinate dehydrogenase (SDH) or mitochondrial complex II is a multimeric enzyme that is bound to the inner membrane of mitochondria and has a dual role as it serves both as a critical step of the tricarboxylic acid or Krebs cycle and as a member of the respiratory chain that transfers electrons directly to the ubiquinone pool. Mutations in SDH subunits have been implicated in the formation of familial paragangliomas (PGLs) and/or pheochromocytomas (PHEOs) and in Carney–Stratakis syndrome. More recently, SDH defects were associated with predisposition to a Cowden disease phenotype, renal, and thyroid cancer. We recently described a kindred with the coexistence of familial PGLs and an aggressive GH-secreting pituitary adenoma, harboring anSDHDmutation. The pituitary tumor showed loss of heterozygosity at theSDHDlocus, indicating the possibility thatSDHD's loss was causatively linked to the development of the neoplasm. In total, 29 cases of pituitary adenomas presenting in association with PHEOs and/or extra-adrenal PGLs have been reported in the literature since 1952. Although a number of other genetic defects are possible in these cases, we speculate that the association of PHEOs and/or PGLs with pituitary tumors is a new syndromic association and a novel phenotype for SDH defects.

CBSX3-Trxo-2 regulates ROS generation of mitochondrial complex II (succinate dehydrogenase) in Arabidopsis

Plant Science ◽  
2020 ◽  
Vol 294 ◽  
pp. 110458 ◽  
Author(s):  
Jin Seok Shin ◽  
Won Mi So ◽  
Soo Youn Kim ◽  
Minsoo Noh ◽  
Sujin Hyoung ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Ros Generation ◽  
Mitochondrial Complex ◽  
Complex Ii

Succinate dehydrogenase (mitochondrial complex II) is a source of reactive oxygen species in plants and regulates development and stress responses

2015 ◽  
Vol 208 (3) ◽  
pp. 776-789 ◽  
Author(s):  
Douglas Jardim-Messeder ◽  
Andréia Caverzan ◽  
Rafael Rauber ◽  
Eduardo de Souza Ferreira ◽  
Márcia Margis-Pinheiro ◽  
...  
Keyword(s):  
Reactive Oxygen Species ◽  
Succinate Dehydrogenase ◽  
Stress Responses ◽  
Reactive Oxygen ◽  
Oxygen Species ◽  
Mitochondrial Complex ◽  
Complex Ii

scholarly journals Synthesis and Antineoplastic Evaluation of Mitochondrial Complex II (Succinate Dehydrogenase) Inhibitors Derived from Atpenin A5

ChemMedChem ◽  
2017 ◽  
Vol 12 (13) ◽  
pp. 1033-1044 ◽  
Author(s):  
Hezhen Wang ◽  
Bader Huwaimel ◽  
Kshitij Verma ◽  
James Miller ◽  
Todd M. Germain ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Mitochondrial Complex ◽  
Complex Ii ◽  
Succinate Dehydrogenase Inhibitors

scholarly journals 15 YEARS OF PARAGANGLIOMA: Genetics and mechanism of pheochromocytoma–paraganglioma syndromes characterized by germline SDHB and SDHD mutations

2015 ◽  
Vol 22 (4) ◽  
pp. T71-T82 ◽  
Author(s):  
Bora E Baysal ◽  
Eamonn R Maher
Keyword(s):  
Pelvic Floor ◽  
Skull Base ◽  
Succinate Dehydrogenase ◽  
Genetic Predisposition ◽  
Neoplastic Transformation ◽  
Neuroendocrine Neoplasms ◽  
Mitochondrial Complex ◽  
Genetic Studies ◽  
Complex Ii ◽  
Induced Genes

Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine neoplasms that derive from small paraganglionic tissues which are located from skull base to the pelvic floor. Genetic predisposition plays an important role in development of PPGLs. Since the discovery of first mutations in the succinate dehydrogenase D (SDHD) gene, which encodes the smallest subunit of mitochondrial complex II (SDH), genetic studies have revealed a major role for mutations in SDH subunit genes, primarily in SDHB and SDHD, in predisposition to both familial and non-familial PPGLs. SDH-mutated PPGLs show robust expression of hypoxia induced genes, and genomic and histone hypermethylation. These effects occur in part through succinate-mediated inhibition of α-ketoglutarate-dependent dioxygenases. However, details of mechanisms by which SDH mutations activate hypoxic pathways and trigger subsequent neoplastic transformation remain poorly understood. Here, we present a brief review of the genetic and mechanistic aspects of SDH-mutated PPGLs.

scholarly journals A Family With a Carotid Body Paraganglioma and Thyroid Neoplasias With a New SDHAF2 Germline Variant

2019 ◽  
Vol 3 (11) ◽  
pp. 2151-2157
Author(s):  
Katherine I Wolf ◽  
Michelle F Jacobs ◽  
Rohit Mehra ◽  
Priya Begani ◽  
Matthew S Davenport ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Carotid Body ◽  
Follicular Adenoma ◽  
Needle Aspiration ◽  
Neck Mass ◽  
Whole Body ◽  
Whole Body Mri ◽  
Pathogenic Variants ◽  
Assembly Factor ◽  
Associated Tumors

Abstract At least 30% of all pheochromocytomas (PCCs)/paragangliomas (PGLs) arise in patients with a germline predisposition syndrome. Variants in succinate dehydrogenase subunits A, B, C, and D (SDHA, SDHB, SDHC, and SDHD) are the most common pathogenic germline alterations. Few pathogenic variants have been reported in succinate dehydrogenase assembly factor 2 (SDHAF2). Here, we describe a 30-year-old female patient who presented with a left-sided neck mass, which was later characterized as a carotid body PGL. Genetic testing revealed a likely pathogenic SDHAF2 variant (c.347G>A;p.W116X). Two sisters carried the same pathologic variant, and screening protocols were recommended. Whole-body MRI revealed thyroid nodules; this testing was followed by fine-needle aspiration, which confirmed papillary thyroid carcinoma in one sister and a follicular adenoma in the other. The two sisters then underwent hemithyroidectomy and total thyroidectomy, respectively. Because evidence for pathogenic variants in SDHAF2 causing predisposition to PCC/PGL is limited, we discuss the challenges in mutational variant interpretation and decision making regarding screening for associated tumors.

scholarly journals COA6 is a mitochondrial complex IV assembly factor critical for biogenesis of mtDNA-encoded COX2

2015 ◽  
Vol 24 (19) ◽  
pp. 5404-5415 ◽  
Author(s):  
David A. Stroud ◽  
Megan J. Maher ◽  
Caroline Lindau ◽  
F.-Nora Vögtle ◽  
Ann E. Frazier ◽  
...  
Keyword(s):  
Mitochondrial Complex ◽  
Complex Iv ◽  
Assembly Factor
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