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Protein

Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial

Gene

SDHB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Iron-sulfur protein (IP) 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).

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

Protein has several cofactor binding sites:

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: tricarboxylic acid cycle

This protein is involved in step 1 of the subpathway that synthesizes fumarate from succinate (eukaryal route).
Proteins known to be involved in this subpathway in this organism are:
  1. Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (SDHA), Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (SDHB), Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial, Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (SDHA), Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (SDHB), Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (SDHA)
This subpathway is part of the pathway tricarboxylic acid cycle, which is itself part of Carbohydrate metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes fumarate from succinate (eukaryal route), the pathway tricarboxylic acid cycle and in Carbohydrate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the ‘Description’ field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi93Iron-sulfur 1 (2Fe-2S)By similarity1
Metal bindingi98Iron-sulfur 1 (2Fe-2S)By similarity1
Metal bindingi101Iron-sulfur 1 (2Fe-2S)By similarity1
Metal bindingi113Iron-sulfur 1 (2Fe-2S)By similarity1
Metal bindingi186Iron-sulfur 2 (4Fe-4S)By similarity1
Metal bindingi189Iron-sulfur 2 (4Fe-4S)By similarity1
Metal bindingi192Iron-sulfur 2 (4Fe-4S)By similarity1
Metal bindingi196Iron-sulfur 3 (3Fe-4S)By similarity1
<p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei201Ubiquinone; shared with DHSDBy similarity1
Metal bindingi243Iron-sulfur 3 (3Fe-4S)By similarity1
Metal bindingi249Iron-sulfur 3 (3Fe-4S)By similarity1
Metal bindingi253Iron-sulfur 2 (4Fe-4S)By similarity1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

  • aerobic respiration Source: GO_Central
  • respiratory electron transport chain Source: GO_Central
  • succinate metabolic process Source: Ensembl
  • tricarboxylic acid cycle Source: ProtInc

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionOxidoreductase
Biological processElectron transport, Transport, Tricarboxylic acid cycle
Ligand2Fe-2S, 3Fe-4S, 4Fe-4S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

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BioCyci
MetaCyc:ENSG00000117118-MONOMER

BRENDA Comprehensive Enzyme Information System

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BRENDAi
1.3.5.1 2681

Reactome - a knowledgebase of biological pathways and processes

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Reactomei
R-HSA-611105 Respiratory electron transport
R-HSA-71403 Citric acid cycle (TCA cycle)

SIGNOR Signaling Network Open Resource

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SIGNORi
P21912

UniPathway: a resource for the exploration and annotation of metabolic pathways

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UniPathwayi
UPA00223;UER01006

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (EC:1.3.5.1)
Alternative name(s):
Iron-sulfur subunit of complex II
Short name:
Ip
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:SDHB
Synonyms:SDH, SDH1
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 1

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000117118.9

Human Gene Nomenclature Database

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HGNCi
HGNC:10681 SDHB

Online Mendelian Inheritance in Man (OMIM)

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MIMi
185470 gene

neXtProt; the human protein knowledge platform

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neXtProti
NX_P21912

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Pheochromocytoma (PCC)7 Publications
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.
See also OMIM:171300
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_03506329A → AQ in PCC. 1 Publication1
Natural variantiVAR_05437643A → P in PCC. 1 Publication1
Natural variantiVAR_03506446R → G in PCC. 3 PublicationsCorresponds to variant dbSNP:rs74315370EnsemblClinVar.1
Natural variantiVAR_05437746R → Q in PCC and PGL4. 3 PublicationsCorresponds to variant dbSNP:rs772551056EnsemblClinVar.1
Natural variantiVAR_05437853G → R in PCC. 1 Publication1
Natural variantiVAR_05437965L → H in PCC. 1 PublicationCorresponds to variant dbSNP:rs876659329EnsemblClinVar.1
Natural variantiVAR_05438065L → P in PCC. 1 PublicationCorresponds to variant dbSNP:rs876659329EnsemblClinVar.1
Natural variantiVAR_01851787L → S in PCC. 2 PublicationsCorresponds to variant dbSNP:rs727504457EnsemblClinVar.1
Natural variantiVAR_037620100S → F in PCC; absence of expression in tumor cells indicating complete loss of SDHB function. 1 PublicationCorresponds to variant dbSNP:rs121917755EnsemblClinVar.1
Natural variantiVAR_035065101C → Y in PCC. 2 PublicationsCorresponds to variant dbSNP:rs74315371EnsemblClinVar.1
Natural variantiVAR_054381127I → N in PCC. 1 Publication1
Natural variantiVAR_035066192C → R in PCC. 2 PublicationsCorresponds to variant dbSNP:rs786202732EnsemblClinVar.1
Natural variantiVAR_035067196C → Y in PCC. 2 PublicationsCorresponds to variant dbSNP:rs876658367EnsemblClinVar.1
Natural variantiVAR_054383230R → C in PCC. 1 PublicationCorresponds to variant dbSNP:rs138996609EnsemblClinVar.1
Natural variantiVAR_017869242R → H in PGL4 and PCC. 3 PublicationsCorresponds to variant dbSNP:rs74315368EnsemblClinVar.1
Paragangliomas 4 (PGL4)5 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.
See also OMIM:115310
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05437746R → Q in PCC and PGL4. 3 PublicationsCorresponds to variant dbSNP:rs772551056EnsemblClinVar.1
Natural variantiVAR_018518131P → R in PGL4. 1 Publication1
Natural variantiVAR_037621132H → P in PGL4. 1 PublicationCorresponds to variant dbSNP:rs74315372EnsemblClinVar.1
Natural variantiVAR_017868197P → R in PGL4. 2 PublicationsCorresponds to variant dbSNP:rs74315367EnsemblClinVar.1
Natural variantiVAR_017869242R → H in PGL4 and PCC. 3 PublicationsCorresponds to variant dbSNP:rs74315368EnsemblClinVar.1
Paraganglioma and gastric stromal sarcoma (PGGSS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionGastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.
See also OMIM:606864

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNET

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DisGeNETi
6390

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

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GeneReviewsi
SDHB

MalaCards human disease database

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MalaCardsi
SDHB
MIMi115310 phenotype
171300 phenotype
606864 phenotype

Open Targets

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OpenTargetsi
ENSG00000117118

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
97286 Carney-Stratakis syndrome
201 Cowden syndrome
44890 Gastrointestinal stromal tumor
29072 Hereditary pheochromocytoma-paraganglioma
3208 Isolated succinate-CoQ reductase deficiency
276624 Sporadic pheochromocytoma

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA35606

Chemistry databases

Drug and drug target database

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DrugBanki
DB00139 Succinic acid
DB08689 UBIQUINONE-1

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
SDHB

Domain mapping of disease mutations (DMDM)

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DMDMi
20455488

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 28MitochondrionCombined sourcesAdd BLAST28
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000001035529 – 280Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrialAdd BLAST252

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei51N6-acetyllysineBy similarity1
Modified residuei55N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
P21912

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
P21912

MaxQB - The MaxQuant DataBase

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MaxQBi
P21912

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
P21912

PeptideAtlas

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PeptideAtlasi
P21912

PRoteomics IDEntifications database

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PRIDEi
P21912

ProteomicsDB human proteome resource

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ProteomicsDBi
53939

2D gel databases

University College Dublin 2-DE Proteome Database

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UCD-2DPAGEi
P21912

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P21912

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
P21912

SwissPalm database of S-palmitoylation events

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SwissPalmi
P21912

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000117118 Expressed in 232 organ(s), highest expression level in muscle of leg

CleanEx database of gene expression profiles

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CleanExi
HS_SDHB

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P21912 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P21912 HS

Organism-specific databases

Human Protein Atlas

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HPAi
CAB009822
CAB068233
CAB068234
CAB068235
HPA002868

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD.

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
112291, 115 interactors

ComplexPortal: manually curated resource of macromolecular complexes

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ComplexPortali
CPX-561 Mitochondrial respiratory chain complex II

Database of interacting proteins

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DIPi
DIP-39666N

Protein interaction database and analysis system

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IntActi
P21912, 33 interactors

Molecular INTeraction database

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MINTi
P21912

STRING: functional protein association networks

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STRINGi
9606.ENSP00000364649

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P21912

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P21912

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini40 – 1332Fe-2S ferredoxin-typePROSITE-ProRule annotationAdd BLAST94
Domaini176 – 2064Fe-4S ferredoxin-typePROSITE-ProRule annotationAdd BLAST31

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG3049 Eukaryota
COG0479 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00390000013558

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000160590

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG005483

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P21912

KEGG Orthology (KO)

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KOi
K00235

Identification of Orthologs from Complete Genome Data

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OMAi
DGQYFGP

Database of Orthologous Groups

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OrthoDBi
507784at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
P21912

TreeFam database of animal gene trees

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TreeFami
TF300754

Family and domain databases

Conserved Domains Database

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CDDi
cd00207 fer2, 1 hit

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
1.10.1060.10, 1 hit
3.10.20.30, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR036010 2Fe-2S_ferredoxin-like_sf
IPR001041 2Fe-2S_ferredoxin-type
IPR006058 2Fe2S_fd_BS
IPR017896 4Fe4S_Fe-S-bd
IPR017900 4Fe4S_Fe_S_CS
IPR012675 Beta-grasp_dom_sf
IPR009051 Helical_ferredxn
IPR004489 Succ_DH/fum_Rdtase_Fe-S
IPR025192 Succ_DH/fum_Rdtase_N

Pfam protein domain database

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Pfami
View protein in Pfam
PF13085 Fer2_3, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF46548 SSF46548, 1 hit
SSF54292 SSF54292, 1 hit

TIGRFAMs; a protein family database

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TIGRFAMsi
TIGR00384 dhsB, 1 hit

PROSITE; a protein domain and family database

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PROSITEi
View protein in PROSITE
PS00197 2FE2S_FER_1, 1 hit
PS51085 2FE2S_FER_2, 1 hit
PS00198 4FE4S_FER_1, 1 hit
PS51379 4FE4S_FER_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All

P21912-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAAVVALSLR RRLPATTLGG ACLQASRGAQ TAAATAPRIK KFAIYRWDPD
60 70 80 90 100
KAGDKPHMQT YEVDLNKCGP MVLDALIKIK NEVDSTLTFR RSCREGICGS
110 120 130 140 150
CAMNINGGNT LACTRRIDTN LNKVSKIYPL PHMYVIKDLV PDLSNFYAQY
160 170 180 190 200
KSIEPYLKKK DESQEGKQQY LQSIEEREKL DGLYECILCA CCSTSCPSYW
210 220 230 240 250
WNGDKYLGPA VLMQAYRWMI DSRDDFTEER LAKLQDPFSL YRCHTIMNCT
260 270 280
RTCPKGLNPG KAIAEIKKMM ATYKEKKASV
Length:280
Mass (Da):31,630
Last modified:May 2, 2002 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iED12E7C3BA7B6D13
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A087WWT1A0A087WWT1_HUMAN
Succinate dehydrogenase [ubiquinone...
SDHB
200Annotation score:

Annotation score:3 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A087WXX8A0A087WXX8_HUMAN
Succinate dehydrogenase [ubiquinone...
SDHB
123Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti19 – 21GGA → WRT in AAA35708 (PubMed:2302193).Curated3
Sequence conflicti19 – 21GGA → WRT in BAA01089 (PubMed:2302193).Curated3
Sequence conflicti62E → K in AAA81167 (PubMed:7622059).Curated1
Sequence conflicti62E → K in AAA35708 (PubMed:2302193).Curated1
Sequence conflicti62E → K in BAA01089 (PubMed:2302193).Curated1
Sequence conflicti67K → NR in AAA80581 (PubMed:7622059).Curated1
Sequence conflicti151K → R no nucleotide entry (PubMed:2494655).Curated1
Sequence conflicti172Q → E no nucleotide entry (PubMed:2494655).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0543743A → G Found in a patient with a Cowden-like phenotype; unknown pathological significance; associated with increased manganese superoxide dismutase expression and normal levels of reactive oxygen species; associated with a 1.2-fold increase in AKT expression and 1.3-fold change in MAPK expression. 1 PublicationCorresponds to variant dbSNP:rs11203289EnsemblClinVar.1
Natural variantiVAR_03506329A → AQ in PCC. 1 Publication1
Natural variantiVAR_05437540K → E1 Publication1
Natural variantiVAR_05437643A → P in PCC. 1 Publication1
Natural variantiVAR_03506446R → G in PCC. 3 PublicationsCorresponds to variant dbSNP:rs74315370EnsemblClinVar.1
Natural variantiVAR_05437746R → Q in PCC and PGL4. 3 PublicationsCorresponds to variant dbSNP:rs772551056EnsemblClinVar.1
Natural variantiVAR_05437853G → R in PCC. 1 Publication1
Natural variantiVAR_05437965L → H in PCC. 1 PublicationCorresponds to variant dbSNP:rs876659329EnsemblClinVar.1
Natural variantiVAR_05438065L → P in PCC. 1 PublicationCorresponds to variant dbSNP:rs876659329EnsemblClinVar.1
Natural variantiVAR_01851787L → S in PCC. 2 PublicationsCorresponds to variant dbSNP:rs727504457EnsemblClinVar.1
Natural variantiVAR_037620100S → F in PCC; absence of expression in tumor cells indicating complete loss of SDHB function. 1 PublicationCorresponds to variant dbSNP:rs121917755EnsemblClinVar.1
Natural variantiVAR_035065101C → Y in PCC. 2 PublicationsCorresponds to variant dbSNP:rs74315371EnsemblClinVar.1
Natural variantiVAR_054381127I → N in PCC. 1 Publication1
Natural variantiVAR_018518131P → R in PGL4. 1 Publication1
Natural variantiVAR_037621132H → P in PGL4. 1 PublicationCorresponds to variant dbSNP:rs74315372EnsemblClinVar.1
Natural variantiVAR_054382163S → P Found in a patient with a Cowden-like phenotype; unknown pathological significance; associated with increased manganese superoxide dismutase function and increased levels of reactive oxygen species; associated with a 2.7-fold change in AKT expression and a 1.7-fold increase in MAPK expression. 1 PublicationCorresponds to variant dbSNP:rs33927012EnsemblClinVar.1
Natural variantiVAR_035066192C → R in PCC. 2 PublicationsCorresponds to variant dbSNP:rs786202732EnsemblClinVar.1
Natural variantiVAR_035067196C → Y in PCC. 2 PublicationsCorresponds to variant dbSNP:rs876658367EnsemblClinVar.1
Natural variantiVAR_017868197P → R in PGL4. 2 PublicationsCorresponds to variant dbSNP:rs74315367EnsemblClinVar.1
Natural variantiVAR_054383230R → C in PCC. 1 PublicationCorresponds to variant dbSNP:rs138996609EnsemblClinVar.1
Natural variantiVAR_017869242R → H in PGL4 and PCC. 3 PublicationsCorresponds to variant dbSNP:rs74315368EnsemblClinVar.1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
U17248 mRNA Translation: AAA81167.1
U17886
, U17296, U17880, U17881, U17882, U17883, U17884, U17885 Genomic DNA Translation: AAA80581.1
AK312056 mRNA Translation: BAG34992.1
AL049569 Genomic DNA No translation available.
CH471134 Genomic DNA Translation: EAW94828.1
BC007840 mRNA Translation: AAH07840.1
DQ403007 mRNA Translation: ABD77140.1
D10245 mRNA Translation: BAA01089.1
M32246 mRNA Translation: AAA35708.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS176.1

Protein sequence database of the Protein Information Resource

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PIRi
I38895

NCBI Reference Sequences

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RefSeqi
NP_002991.2, NM_003000.2

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.465924

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000375499; ENSP00000364649; ENSG00000117118

Database of genes from NCBI RefSeq genomes

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GeneIDi
6390

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:6390

UCSC genome browser

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UCSCi
uc001bae.5 human

Keywords - Coding sequence diversityi

Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology
TCA Cycle Gene Mutation Database

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U17248 mRNA Translation: AAA81167.1
U17886
, U17296, U17880, U17881, U17882, U17883, U17884, U17885 Genomic DNA Translation: AAA80581.1
AK312056 mRNA Translation: BAG34992.1
AL049569 Genomic DNA No translation available.
CH471134 Genomic DNA Translation: EAW94828.1
BC007840 mRNA Translation: AAH07840.1
DQ403007 mRNA Translation: ABD77140.1
D10245 mRNA Translation: BAA01089.1
M32246 mRNA Translation: AAA35708.1
CCDSiCCDS176.1
PIRiI38895
RefSeqiNP_002991.2, NM_003000.2
UniGeneiHs.465924

3D structure databases

ProteinModelPortaliP21912
SMRiP21912
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi112291, 115 interactors
ComplexPortaliCPX-561 Mitochondrial respiratory chain complex II
DIPiDIP-39666N
IntActiP21912, 33 interactors
MINTiP21912
STRINGi9606.ENSP00000364649

Chemistry databases

DrugBankiDB00139 Succinic acid
DB08689 UBIQUINONE-1

PTM databases

iPTMnetiP21912
PhosphoSitePlusiP21912
SwissPalmiP21912

Polymorphism and mutation databases

BioMutaiSDHB
DMDMi20455488

2D gel databases

UCD-2DPAGEiP21912

Proteomic databases

EPDiP21912
jPOSTiP21912
MaxQBiP21912
PaxDbiP21912
PeptideAtlasiP21912
PRIDEiP21912
ProteomicsDBi53939

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
6390
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000375499; ENSP00000364649; ENSG00000117118
GeneIDi6390
KEGGihsa:6390
UCSCiuc001bae.5 human

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
6390
DisGeNETi6390
EuPathDBiHostDB:ENSG00000117118.9

GeneCards: human genes, protein and diseases

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GeneCardsi
SDHB
GeneReviewsiSDHB
HGNCiHGNC:10681 SDHB
HPAiCAB009822
CAB068233
CAB068234
CAB068235
HPA002868
MalaCardsiSDHB
MIMi115310 phenotype
171300 phenotype
185470 gene
606864 phenotype
neXtProtiNX_P21912
OpenTargetsiENSG00000117118
Orphaneti97286 Carney-Stratakis syndrome
201 Cowden syndrome
44890 Gastrointestinal stromal tumor
29072 Hereditary pheochromocytoma-paraganglioma
3208 Isolated succinate-CoQ reductase deficiency
276624 Sporadic pheochromocytoma
PharmGKBiPA35606

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG3049 Eukaryota
COG0479 LUCA
GeneTreeiENSGT00390000013558
HOGENOMiHOG000160590
HOVERGENiHBG005483
InParanoidiP21912
KOiK00235
OMAiDGQYFGP
OrthoDBi507784at2759
PhylomeDBiP21912
TreeFamiTF300754

Enzyme and pathway databases

UniPathwayi
UPA00223;UER01006

BioCyciMetaCyc:ENSG00000117118-MONOMER
BRENDAi1.3.5.1 2681
ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-71403 Citric acid cycle (TCA cycle)
SIGNORiP21912

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
SDHB human

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
SDHB

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
6390

Protein Ontology

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PROi
PR:P21912

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

Gene expression databases

BgeeiENSG00000117118 Expressed in 232 organ(s), highest expression level in muscle of leg
CleanExiHS_SDHB
ExpressionAtlasiP21912 baseline and differential
GenevisibleiP21912 HS

Family and domain databases

CDDicd00207 fer2, 1 hit
Gene3Di1.10.1060.10, 1 hit
3.10.20.30, 1 hit
InterProiView protein in InterPro
IPR036010 2Fe-2S_ferredoxin-like_sf
IPR001041 2Fe-2S_ferredoxin-type
IPR006058 2Fe2S_fd_BS
IPR017896 4Fe4S_Fe-S-bd
IPR017900 4Fe4S_Fe_S_CS
IPR012675 Beta-grasp_dom_sf
IPR009051 Helical_ferredxn
IPR004489 Succ_DH/fum_Rdtase_Fe-S
IPR025192 Succ_DH/fum_Rdtase_N
PfamiView protein in Pfam
PF13085 Fer2_3, 1 hit
SUPFAMiSSF46548 SSF46548, 1 hit
SSF54292 SSF54292, 1 hit
TIGRFAMsiTIGR00384 dhsB, 1 hit
PROSITEiView protein in PROSITE
PS00197 2FE2S_FER_1, 1 hit
PS51085 2FE2S_FER_2, 1 hit
PS00198 4FE4S_FER_1, 1 hit
PS51379 4FE4S_FER_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiSDHB_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P21912
Secondary accession number(s): B2R545, Q0QEY7, Q9NQ12
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 1, 1991
Last sequence update: May 2, 2002
Last modified: January 16, 2019
This is version 215 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
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