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SDHB succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [Homo sapiens (human)]


RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_003000 Homo sapiens succinate dehydrogenase complex, subunit B, iron sulfur (Ip) (SDHB), mRNA. GenEZ ORF Cloning On-demand $449.00 14

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Gene Symbol SDHB
Entrez Gene ID 6390
Full Name succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Synonyms CWS2, IP, PGL4, SDH, SDH1, SDH2, SDHIP
General protein information
Preferred Names
succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
Names
succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
iron-sulfur subunit of complex II
NP_002991.2
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

1

1p36.1-p35

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 iron-sulfur subunit is highly conserved and contains three cysteine-rich clusters which may comprise the iron-sulfur centers of the enzyme. Sporadic and familial mutations in this gene result in paragangliomas and pheochromocytoma, and support a link between mitochondrial dysfunction and tumorigenesis. [provided by RefSeq, Jul 2008].
Disorder

MIM: 185470

Paraganglioma, familial chromaffin, 4, 115310 (3); Pheochromocytoma,

mRNA Protein Product Sequence Price Select
NM_003000, 115387093 NP_002991, 115387094 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor ORF Sequence $300.00
KEGG
hsa00020Citrate cycle (TCA cycle)
hsa00190Oxidative phosphorylation
hsa05016Huntington's disease
hsa05012Parkinson's disease
hsa05010Alzheimer's disease
hsa_M00148Succinate dehydrogenase (ubiquinone)
hsa_M00011Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate
hsa01200Carbon metabolism
hsa_M00009Citrate cycle (TCA cycle, Krebs cycle)
hsa04932Non-alcoholic fatty liver disease (NAFLD)
WikiPathways
WP78TCA Cycle
WP111Electron Transport Chain
BIOCYC
HUMAN_PWY-5690TCA cycle II (eukaryotic)
META_PWY66-398TCA cycle VIII (metazoan)
Reactome
REACT_6305Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.
REACT_1785Citric acid cycle (TCA cycle)
REACT_1046Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_22393Respiratory electron transport
REACT_111217Metabolism
REACT_111083The citric acid (TCA) cycle and respiratory electron transport
Homo sapiens (human)SDHBNP_002991.2
Pan troglodytes (chimpanzee)SDHBXP_003949355.1
Macaca mulatta (Rhesus monkey)SDHBXP_001088349.1
Canis lupus familiaris (dog)SDHBNP_001239146.1
Bos taurus (cattle)SDHBNP_001035573.1
Mus musculus (house mouse)SdhbNP_075863.2
Rattus norvegicus (Norway rat)SdhbNP_001094009.1
Gallus gallus (chicken)SDHBXP_004950147.1
Danio rerio (zebrafish)sdhbNP_001092210.1
Drosophila melanogaster (fruit fly)SdhBNP_477101.1
Caenorhabditis eleganssdhb-1NP_495992.1
Arabidopsis thaliana (thale cress)SDH2-1NP_001118718.1
Arabidopsis thaliana (thale cress)SDH2-2NP_198881.1
Xenopus (Silurana) tropicalis (western clawed frog)sdhbNP_001120000.1
Process
IDNameEvidence
GO:0006099tricarboxylic acid cycleIEA
GO:0006099tricarboxylic acid cycleTAS
GO:0006105succinate metabolic processIEA
GO:0009060aerobic respirationTAS
GO:0022904respiratory electron transport chainTAS
GO:0044237cellular metabolic processTAS
GO:0044281small molecule metabolic processTAS
Component
IDNameEvidence
GO:0005739mitochondrionTAS
GO:0005743mitochondrial inner membraneISS
GO:0005743mitochondrial inner membraneTAS
GO:0005749mitochondrial respiratory chain complex IIISS
GO:0070062extracellular vesicular exosomeIDA
Function
IDNameEvidence
GO:0005515protein bindingIPI
GO:0008177succinate dehydrogenase (ubiquinone) activityIEA
GO:0009055electron carrier activityIEA
GO:0046872metal ion bindingIEA
GO:0048039ubiquinone bindingISS
GO:00515372 iron, 2 sulfur cluster bindingISS
GO:00515383 iron, 4 sulfur cluster bindingISS
GO:00515394 iron, 4 sulfur cluster bindingISS
GeneCards SDHB
UniProt P21912
Vega OTTHUMG00000002289
MIM 185470
Ensembl ENSG00000117118
HGNC 10681
HPRD 01707

GeneRIFs: Gene References Into Functions What's a GeneRIF?

What is the normal function of the SDHB gene?

The SDHB gene provides instructions for making one of four subunits of the succinate dehydrogenase (SDH) enzyme. The SDH enzyme plays a critical role in mitochondria, which are structures inside cells that convert the energy from food into a form that cells can use.

Within mitochondria, the SDH enzyme links two important pathways in energy conversion: the citric acid cycle (or Krebs cycle) and oxidative phosphorylation. As part of the citric acid cycle, the SDH enzyme converts a compound called succinate to another compound called fumarate. Negatively charged particles called electrons are released during this reaction. The SDHB protein provides an attachment site for electrons as they are transferred to the oxidative phosphorylation pathway. In oxidative phosphorylation, the electrons help create an electrical charge that provides energy for the production of adenosine triphosphate (ATP), the cell's main energy source.

Succinate, the compound on which the SDH enzyme acts, is an oxygen sensor in the cell and can help turn on specific pathways that stimulate cells to grow in a low-oxygen environment (hypoxia). In particular, succinate stabilizes a protein called hypoxia-inducible factor (HIF) by preventing a reaction that would allow HIF to be broken down. HIF controls several important genes involved in cell division and the formation of new blood vessels in a hypoxic environment.

The SDHB gene is a tumor suppressor, which means it prevents cells from growing and dividing in an uncontrolled way.

Interactant

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