mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)
Biological process
tricarboxylic acid cycle
mitochondrial electron transport, succinate to ubiquinone
Sources:Amigo / QuickGO
Orthologs
Species
Human
Mouse
Entrez
6392
66925
Ensembl
ENSG00000204370
ENSMUSG00000000171
UniProt
O14521
Q9CXV1
RefSeq (mRNA)
NM_001276503 NM_001276504 NM_001276506 NM_003002
NM_025848
RefSeq (protein)
NP_001263432 NP_001263433 NP_001263435 NP_002993
NP_080124
Location (UCSC)
Chr 11: 112.09 – 112.12 Mb
Chr 9: 50.51 – 50.52 Mb
PubMed search
[3]
[4]
Wikidata
View/Edit Human
View/Edit Mouse
Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial (CybS), also known as succinate dehydrogenase complex subunit D (SDHD), is a protein that in humans is encoded by the SDHD gene. Names previously used for SDHD were PGL and PGL1. Succinate dehydrogenase is an important enzyme in both the citric acid cycle and the electron transport chain.[5][6][7] Hereditary PGL-PCC syndrome is caused by a parental imprint of the SDHD gene. Screening can begin by 6 years of age.
^ abcGRCh38: Ensembl release 89: ENSG00000204370 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000000171 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Entrez Gene: succinate dehydrogenase complex".
^Heutink P, van der Mey AG, Sandkuijl LA, van Gils AP, Bardoel A, Breedveld GJ, van Vliet M, van Ommen GJ, Cornelisse CJ, Oostra BA (April 1992). "A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 11q23-qter". Human Molecular Genetics. 1 (1): 7–10. doi:10.1093/hmg/1.1.7. PMID 1301144.
^Hirawake H, Taniwaki M, Tamura A, Kojima S, Kita K (1997). "Cytochrome b in human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the components in liver mitochondria and chromosome assignment of the genes for the large (SDHC) and small (SDHD) subunits to 1q21 and 11q23". Cytogenetics and Cell Genetics. 79 (1–2): 132–8. doi:10.1159/000134700. PMID 9533030.
dehydrogenase complex subunit D (SDHD), is a protein that in humans is encoded by the SDHD gene. Names previously used for SDHD were PGL and PGL1. Succinate...
The second two subunits are hydrophobic membrane anchor subunits, SdhC and SdhD. Human mitochondria contain two distinct isoforms of SdhA (Fp subunits type...
inner mitochondrial membrane. The other transmembrane subunit is SDHD. The SDHC/SDHD dimer is connected to the SDHB electron transport subunit which,...
SDHD. The SDHB subunit is connected to the SDHA subunit on the hydrophilic, catalytic end of the SDH complex. It is also connected to the SDHC/SDHD subunits...
an earlier age). Mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been...
(mutations) in a gene responsible for the enzyme succinate dehydrogenase (SDHD). Temporal bone fractures were historically divided into three main categories...
closely surrounded by the following genes on chromosome 11. DLAT PIH1D2 TIMM8B SDHD The human NKAPD1 gene is ubiquitously expressed at moderate levels in various...
complex subunit C (SDHC); and succinate dehydrogenase complex subunit D (SDHD). Other electron donors (e.g., fatty acids and glycerol 3-phosphate) also...
patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis". The Lancet. Oncology...
suppressor gene pathway involving the succinate dehydrogenase (SDH) subunits SDHD, SDHC and SDHB. Approximately 85% GISTs are associated with an abnormal c-KIT...
responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). SDHD NM_003001 HADHA Trifunctional protein subunit alpha COMT Catechol-O-methyl...
in the SDHC/SDHD dimer. The O1 carbonyl oxygen of ubiquinone is oriented at the active site by hydrogen bond interactions with Tyr83 of SDHD. The presence...
(succinate:ubiquinone oxidoreductase) subunit genes (SDHD, SDHAF2, SDHC, SDHB). PGL-1 is associated with SDHD mutation, and most PGL-1 individuals with paraganglioma...
Paraganglioma, a type of rare neuroendocrine neoplasm The succinate dehydrogenase SDHD (previously known as PGL1) and SDHC (previously PGL3) This disambiguation...
or SDHAF1, but other pathogenic mutations for CII are known for SDHB and SDHD. SDHA is the only SDHx gene reported to date in which dominant pathogenic...
reductase deficiency; succinate dehydrogenase deficiency) SDHA, SDHAF1, SDHD, SDHB AR PS252011 cytochrome b of complex III (MTCYB); Exercise intolerance;...
complementary to the junction between the second and third genes of the sdh operon (sdhD and sdhA). Secondary structure predictions have indicated that this interaction...
proteins that make up the succinate dehydrogenase heterotetrmer, i.e., the SDHD, SDHA, SDHC, and SDHB genes (inactivation of any one of these genes causes...
Söderkvist P, Dahia PL, Neumann HP, Gimm O (March 2014). "Role of SDHAF2 and SDHD in von Hippel-Lindau associated pheochromocytomas". World Journal of Surgery...
Devilee P, Devlin B (Feb 2001). "A high-resolution integrated map spanning the SDHD gene at 11q23: a 1.1-Mb BAC contig, a partial transcript map and 15 new repeat...
Devilee P, Devlin B (2001). "A high-resolution integrated map spanning the SDHD gene at 11q23: a 1.1-Mb BAC contig, a partial transcript map and 15 new repeat...
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