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SDHD succinate dehydrogenase complex, subunit D, integral membrane protein [Homo sapiens (human)]


RefSeq Accession Definition Sequence Price Select
NM_003002 Homo sapiens succinate dehydrogenase complex, subunit D, integral membrane protein (SDHD), nuclear gene encoding mitochondrial protein, mRNA. Full Length $400.78
ORF Sequence $159.00


Gene Symbol SDHD
Entrez Gene ID 6392
Full Name succinate dehydrogenase complex, subunit D, integral membrane protein
Synonyms CBT1, PGL, PGL1, SDH4
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

11

11q23
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. [provided by RefSeq].
Disorder

MIM: 602690

Paragangliomas, familial nonchromaffin, 1, with or without deafness,
mRNA NM_003002, 222352156
Protein NP_002993, 4506865
Product succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial precursor
RefSeq Status REVIEWED
KEGG
hsa00020Citrate cycle (TCA cycle)
hsa05016Huntington's disease
hsa05012Parkinson's disease
hsa01100Metabolic pathways
hsa00190Oxidative phosphorylation
hsa05010Alzheimer's disease
WikiPathways
WP111Electron Transport Chain
WP78TCA Cycle
BIOCYC
META_PWY-5690TCA cycle variation III (eukaryotic)
Reactome
REACT_1785Citric acid cycle (TCA cycle)
REACT_22393Respiratory electron transport
REACT_1046Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_6305Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.
Homo sapiens (human)SDHDNP_002993.1
Pan troglodytes (chimpanzee)SDHDXP_508756.1
Canis lupus familiaris (dog)LOC479438XP_536573.2
Mus musculus (house mouse)SdhdNP_080124.1
Rattus norvegicus (Norway rat)SdhdNP_942083.1
Gallus gallus (chicken)SDHDNP_001006321.1
Danio rerio (zebrafish)sdhdbNP_001002489.1
Danio rerio (zebrafish)sdhdaNP_001004004.1
Drosophila melanogaster (fruit fly)CG10219NP_651181.1
Process
IDNameEvidence
GO:0006099tricarboxylic acid cycleIDA
GO:0006099tricarboxylic acid cycleTAS
GO:0006810transportIEA
GO:0022904respiratory electron transport chainTAS
Component
IDNameEvidence
GO:0005739mitochondrionIEA
GO:0005740mitochondrial envelopeIEA
GO:0005743mitochondrial inner membraneEXP
GO:0005743mitochondrial inner membraneIDA
GO:0005743mitochondrial inner membraneISS
GO:0005749mitochondrial respiratory chain complex IIISS
GO:0016020membraneIEA
GO:0016021integral to membraneIEA
Function
IDNameEvidence
GO:0000104succinate dehydrogenase activityIDA
GO:0009055electron carrier activityTAS
GO:0020037heme bindingISS
GO:0046872metal ion bindingIEA
GO:0048039ubiquinone bindingISS
GeneCards SDHD
UniProt O14521
MIM 602690
Ensembl ENSG00000204370
HGNC 10683
HPRD 04069

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