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

Summary

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Official Symbol: SDHBprovided by HGNC
Official Full Name: succinate dehydrogenase complex, subunit B, iron sulfur (Ip)provided by HGNC
Primary source: HGNC:10681
See related: Ensembl:ENSG00000117118; HPRD:01707; MIM:185470; Vega:OTTHUMG00000002289
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Homo sapiens
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as: IP; SDH; CWS2; PGL4; SDH1; SDH2; SDHIP
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]

Genomic context

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Location :: 1p36.1-p35

Sequence :: Chromosome: 1; NC_000001.10 (17345217..17380665, complement)

See SDHB in Epigenomics, MapViewer

Chromosome 1 - NC_000001.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence

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Go to reference sequence details

Bibliography

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

This report represents the first example of SDHB mutation as a cause of inherited mitochondrial respiratory chain disease and extends the SDHA mutation spectrum in patients with isolated complex II deficiency.
Title: Recessive germline SDHA and SDHB mutations causing leukodystrophy and isolated mitochondrial complex II deficiency.
All SDHB-deficient GISTs with known primary sites arose in the stomach, and had multinodular architecture and epithelioid or mixed morphology.
Title: Loss of succinate dehydrogenase subunit B (SDHB) expression is limited to a distinctive subset of gastric wild-type gastrointestinal stromal tumours: a comprehensive genotype-phenotype correlation study.
These findings provide the first direct mechanism of functional loss resulting from SDHB mutations and suggest that with histone deacetylase inhibitors may serve as a therapeutic paradigm for preventing the development of SDHB-related tumors.
Title: Missense mutations in the human SDHB gene increase protein degradation without altering intrinsic enzymatic function.
Succinate dehydrogenase-deficient gastro-intestinal stromal tumors are characterized by IGF1R overexpression.
Title: Succinate dehydrogenase-deficient GISTs are characterized by IGF1R overexpression.
8 different SDHB mutations were found in 10 patients with sporadic paraganglioma.
Title: Prevalence and spectrum of SDHx mutations in pheochromocytoma and paraganglioma in patients from Belgium: an update.
a novel germline SDHB mutation (c.T282A--Ile44Asn) occurring in a gastrointestinal stromal tumor patient
Title: A novel germline SDHB mutation in a gastrointestinal stromal tumor patient without bona fide features of the Carney-Stratakis dyad.
Tumor DNA mutation screening of the SDHx, VHL, and RET genes and LOH analyses of the SDHx and VHL genes were performed for pheochromocytoma.
Title: Somatic mutation analysis of the SDHB, SDHC, SDHD, and RET genes in the clinical assessment of sporadic and hereditary pheochromocytoma.
The high frequency of founder mutations in SDHB suggests a higher prevalence of malignancy, and the SDHD mutation is usually associated with familial cases.
Title: Genetic and clinical characteristics of head and neck paragangliomas in a Chinese population.
Analysis of germline mutation frequencies revealed two cases with missense, one case with a splice-site mutation, and six cases of single nucleotide polymorphisms in SDHB gene in pheochromocytoma and abdominal paraganglioma in Western Sweden.
Title: Prevalence of germline mutations in patients with pheochromocytoma or abdominal paraganglioma and sporadic presentation: a population-based study in Western Sweden.
Tumor-derived FH and SDH mutations accumulate fumarate and succinate, leading to enzymatic inhibition of multiple alpha-KG-dependent dioxygenases and consequent alterations of genome-wide histone and DNA methylation.
Title: Inhibition of α-KG-dependent histone and DNA demethylases by fumarate and succinate that are accumulated in mutations of FH and SDH tumor suppressors.

Submit: New GeneRIF Correction See all (82)

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

Gastrointestinal Stromal Tumors

MIM: 606764

Genetic Testing Registry

Paraganglioma and gastric stromal sarcoma

MIM: 606864

Genetic Testing Registry

Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base, neck, and upper medistinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically hypersecrete catecholamines. Symptoms of PGL/PCC result either from mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for malignant transformation is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas or skull base and neck paragangliomas.

Diagnosis Testing

The diagnosis of hereditary PGL/PCC syndromes is based on physical examination, family history, imaging studies, biochemical testing, and molecular genetic testing. SDHA, SDHB, SDHC and SDHD are four nuclear genes that encode the four subunits of the mitochondrial enzyme succinate dehydrogenase (SDH). A fifth nuclear gene, SDHAF2 (also known as SDH5) encodes a protein that appears to be required for flavination of another SDH subunit, SDHA. These are collectively known as the SDHx genes. Mutations in MAX predispose to PCC; a subset of individuals with mutations in MAX will also develop PGL. KIF1B and EGLN1 (formerly known as PHD2) have been reported to be associated with hereditary PGL/PCC, but their clinical significance is still unclear. Molecular genetic testing for disease-causing variants in SDHA, SDHB, SDHC, SDHD, SDHAF2, and MAX is clinically available.

Genetic Counseling

The hereditary PGL/PCC syndromes are inherited in an autosomal dominant manner. Mutations in SDHD (PGL1) demonstrate parent-of-origin effects and generally cause disease only when the mutation is inherited from the father. Initial data suggest that mutations in SDHAF2 (PGL2) and MAX exhibit parent-of-origin effects similar to those of mutations in SDHD. A proband with a hereditary PGL/PCC syndrome may have inherited the mutation from a parent or have a de novo mutation; the proportion of cases caused by de novo mutations is unknown. Each child of an individual with a hereditary PGL/PCC syndrome has a 50% chance of inheriting the disease-causing mutation. An individual who inherits a SDHD mutation from his/her mother is at a low but not negligible risk of developing disease; each of his/her offspring is at a 50% risk of inheriting the disease-causing allele. An individual who inherits an SDHD mutation from his/her father is at high risk of manifesting paragangliomas and, to a lesser extent, pheochromocytomas. Prenatal testing for pregnancies at increased risk is possible for families in which the disease-causing mutation is known; if no laboratories offering prenatal testing are listed in the GeneTeststrade mark Laboratory Directory, such testing may be available through laboratories offering custom prenatal testing.

References

PMID: 20301715

Pheochromocytoma

MIM: 171300

Genetic Testing Registry

Summary from GeneReviews: Hereditary Paraganglioma-Pheochromocytoma Syndromes

Disease Characteristics

Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base, neck, and upper medistinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically hypersecrete catecholamines. Symptoms of PGL/PCC result either from mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for malignant transformation is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas or skull base and neck paragangliomas.

Diagnosis Testing

The diagnosis of hereditary PGL/PCC syndromes is based on physical examination, family history, imaging studies, biochemical testing, and molecular genetic testing. SDHA, SDHB, SDHC and SDHD are four nuclear genes that encode the four subunits of the mitochondrial enzyme succinate dehydrogenase (SDH). A fifth nuclear gene, SDHAF2 (also known as SDH5) encodes a protein that appears to be required for flavination of another SDH subunit, SDHA. These are collectively known as the SDHx genes. Mutations in MAX predispose to PCC; a subset of individuals with mutations in MAX will also develop PGL. KIF1B and EGLN1 (formerly known as PHD2) have been reported to be associated with hereditary PGL/PCC, but their clinical significance is still unclear. Molecular genetic testing for disease-causing variants in SDHA, SDHB, SDHC, SDHD, SDHAF2, and MAX is clinically available.

Genetic Counseling

The hereditary PGL/PCC syndromes are inherited in an autosomal dominant manner. Mutations in SDHD (PGL1) demonstrate parent-of-origin effects and generally cause disease only when the mutation is inherited from the father. Initial data suggest that mutations in SDHAF2 (PGL2) and MAX exhibit parent-of-origin effects similar to those of mutations in SDHD. A proband with a hereditary PGL/PCC syndrome may have inherited the mutation from a parent or have a de novo mutation; the proportion of cases caused by de novo mutations is unknown. Each child of an individual with a hereditary PGL/PCC syndrome has a 50% chance of inheriting the disease-causing mutation. An individual who inherits a SDHD mutation from his/her mother is at a low but not negligible risk of developing disease; each of his/her offspring is at a 50% risk of inheriting the disease-causing allele. An individual who inherits an SDHD mutation from his/her father is at high risk of manifesting paragangliomas and, to a lesser extent, pheochromocytomas. Prenatal testing for pregnancies at increased risk is possible for families in which the disease-causing mutation is known; if no laboratories offering prenatal testing are listed in the GeneTeststrade mark Laboratory Directory, such testing may be available through laboratories offering custom prenatal testing.

References

PMID: 20301715

NHGRI GWAS Catalog

A genome-wide association study in 19 633 Japanese subjects identified LHX3-QSOX2 and IGF1 as adult height loci.

HIV-1 protein interactions

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Protein	Gene	Interaction	Pubs
Tat, p14	tat	Using a yeast two-hybrid system, HIV-1 Tat has been shown to bind the human succinate-ubiquinone oxidoreductase iron sulfur subunit, suggesting a role for this protein in mediating the biological activity of Tat during HIV-1 replication	PubMed

Go to the HIV-1, Human Protein Interaction Database

Interactions

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Products	Interactant	Other Gene	Source	Pubs	Description
BioGRID:112291	BioGRID:106537	ABCA1	BioGRID	PubMed	Two-hybrid
BioGRID:112291	BioGRID:106556	ACAT1	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:106994	ATP5B	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:120937	CAND1	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:116183	COPS5	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:114032	CUL1	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:114031	CUL2	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:114030	CUL3	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:108937	GEM	BioGRID	PubMed	Two-hybrid
BioGRID:112291	BioGRID:109189	GSN	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:128685	HNRNPUL2	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:114353	IQGAP1	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:109883	ITGA4	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:120807	IWS1	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:115114	KIAA0101	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:118783	LAMTOR2	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:120374	MRPL20	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:122364	MRPL40	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:124037	MRPL45	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:120472	MRPS10	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:122362	MRPS5	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:110785	NDUFA10	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:110784	NDUFA9	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:120026	NDUFB11	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:110792	NDUFB6	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:110795	NDUFB9	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:110799	NDUFS1	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:110807	NDUFV2	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:120280	OCIAD1	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:111058	OXA1L	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:125082	PIGS	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:117079	PIK3R5	BioGRID	PubMed	Two-hybrid
BioGRID:112291	BioGRID:115700	RBM14	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:112290	SDHA	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:122862	SLC25A22	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:115547	SPRY2	BioGRID	PubMed	Two-hybrid
BioGRID:112291	BioGRID:112620	SSBP1	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:124961	TIMM50	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:116158	TMED2	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:115716	TOMM40	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:120030	TOMM7	BioGRID	PubMed	Co-fractionation
BioGRID:112291	BioGRID:113011	TP53BP1	BioGRID	PubMed	Affinity Capture-Western; Two-hybrid
BioGRID:112291	BioGRID:113164	UBC	BioGRID	PubMed	Affinity Capture-MS
BioGRID:112291	BioGRID:113232	UQCRFS1	BioGRID	PubMed	Co-fractionation

Pathways from BioSystems

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Alzheimer's disease, organism-specific biosystem (from KEGG)
Alzheimer's disease, organism-specific biosystemAlzheimer's disease (AD) is a chronic disorder that slowly destroys neurons and causes serious cognitive disability. AD is associated with senile plaques and neurofibrillary tangles (NFTs). Amyloid-b...
Alzheimer's disease, conserved biosystem (from KEGG)
Alzheimer's disease, conserved biosystemAlzheimer's disease (AD) is a chronic disorder that slowly destroys neurons and causes serious cognitive disability. AD is associated with senile plaques and neurofibrillary tangles (NFTs). Amyloid-b...
Citrate cycle (TCA cycle), organism-specific biosystem (from KEGG)
Citrate cycle (TCA cycle), organism-specific biosystemThe citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form ...
Citrate cycle (TCA cycle), conserved biosystem (from KEGG)
Citrate cycle (TCA cycle), conserved biosystemThe citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form ...
Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate, organism-specific biosystem (from KEGG)
Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate, organism-specific biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate, conserved biosystem (from KEGG)
Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate, conserved biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
Citric acid cycle (TCA cycle), organism-specific biosystem (from REACTOME)
Citric acid cycle (TCA cycle), organism-specific biosystemIn the citric acid or tricarboxylic acid (TCA) cycle, the acetyl group of acetyl CoA (derived primarily from oxidative decarboxylation of pyruvate, beta-oxidation of long-chain fatty acids, and catab...
Electron Transport Chain, organism-specific biosystem (from WikiPathways)
Electron Transport Chain, organism-specific biosystemAn electron transport chain(ETC) couples a chemical reaction between an electron donor (such as NADH) and an electron acceptor (such as O2) to the transfer of H+ ions across a membrane, through a set...
Huntington's disease, organism-specific biosystem (from KEGG)
Huntington's disease, organism-specific biosystemHuntington disease (HD) is an autosomal-dominant neurodegenerative disorder that primarily affects medium spiny striatal neurons (MSN). The symptoms are choreiform, involuntary movements, personality...
Huntington's disease, conserved biosystem (from KEGG)
Huntington's disease, conserved biosystemHuntington disease (HD) is an autosomal-dominant neurodegenerative disorder that primarily affects medium spiny striatal neurons (MSN). The symptoms are choreiform, involuntary movements, personality...
Metabolism, organism-specific biosystem (from REACTOME)
Metabolism, organism-specific biosystemMetabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as th...
Oxidative phosphorylation, organism-specific biosystem (from KEGG)
Oxidative phosphorylation, organism-specific biosystem
Oxidative phosphorylation
Oxidative phosphorylation, conserved biosystem (from KEGG)
Oxidative phosphorylation, conserved biosystem
Oxidative phosphorylation
Parkinson's disease, organism-specific biosystem (from KEGG)
Parkinson's disease, organism-specific biosystemParkinson's disease (PD) is a progressive neurodegenerative movement disorder that results primarily from the death of dopaminergic neurons in the substantia nigra. Mutations in alpha-synuclein, UCHL...
Pyruvate metabolism and Citric Acid (TCA) cycle, organism-specific biosystem (from REACTOME)
Pyruvate metabolism and Citric Acid (TCA) cycle, organism-specific biosystemPyruvate metabolism and the citric acid (TCA) cycle together link the processes of energy metabolism in a human cell with one another and with key biosynthetic reactions. Pyruvate, derived from the r...
Respiratory electron transport, organism-specific biosystem (from REACTOME)
Respiratory electron transport, organism-specific biosystemMitochondria are often described as the "powerhouse" of a cell as it is here that energy is largely released from the oxidation of food. Reducing equivalents generated from beta-oxidation of fatty ac...
Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins., organism-specific biosystem (from REACTOME)
Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins., organism-specific biosystemOxidation of fatty acids and pyruvate in the mitochondrial matrix yield large amounts of NADH. The respiratory electron transport chain couples the re-oxidation of this NADH to NAD+ to the export of ...
Succinate dehydrogenase (ubiquinone), organism-specific biosystem (from KEGG)
Succinate dehydrogenase (ubiquinone), organism-specific biosystemStructural complex; Energy metabolism; ATP synthesis
TCA Cycle, organism-specific biosystem (from WikiPathways)
TCA Cycle, organism-specific biosystemThe [[wikipedia:citric_acid_cycle|citric acid cycle]], also known as the tricarboxylic acid cycle (TCA cycle) or the Krebs cycle, (or rarely, the Szent-Gyorgyi-Krebs cycle) is a series of enzyme-cata...
TCA cycle, organism-specific biosystem (from BIOCYC)
TCA cycle, organism-specific biosystem
TCA cycle
TCA cycle II (eukaryotic), conserved biosystem (from BIOCYC)
TCA cycle II (eukaryotic), conserved biosystemGeneral Background The TCA pathway is a catabolic pathway of aerobic respiration that generates both energy and reducing power. In addition, it is also the first step in generating precursors for bi...
The citric acid (TCA) cycle and respiratory electron transport, organism-specific biosystem (from REACTOME)
The citric acid (TCA) cycle and respiratory electron transport, organism-specific biosystemThe metabolism of pyruvate provides one source of acetyl-CoA which enters the citric acid (TCA, tricarboxylic acid) cycle to generate energy and the reducing equivalent NADH. These reducing equivalen...

General gene information

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Markers

SHGC-74296 (e-PCR)
- UniSTS:72501

Genotypes

See SDHB SNP Geneview Report
See SDHB SNP Genotype Report
See SDHB SNP Variation Viewer Report

Homology

Homologs of the SDHB gene: The SDHB gene is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, rat, chicken, zebrafish, fruit fly, mosquito, C.elegans, S.cerevisiae, K.lactis, E.gossypii, S.pombe, M.oryzae, N.crassa, A.thaliana, and rice.
Map Viewer (Mouse, Rat)
OrthoDB: The Hierarchical Catalog of Eukaryotic Orthologs

Clone Names

FLJ92337

Gene Ontology Provided by GOA

Function	Evidence Code	Pubs
2 iron, 2 sulfur cluster binding	ISS Inferred from Sequence or Structural Similarity more info
3 iron, 4 sulfur cluster binding	ISS Inferred from Sequence or Structural Similarity more info
4 iron, 4 sulfur cluster binding	ISS Inferred from Sequence or Structural Similarity more info
electron carrier activity	IEA Inferred from Electronic Annotation more info
metal ion binding	IEA Inferred from Electronic Annotation more info
protein binding	IPI Inferred from Physical Interaction more info	PubMed
succinate dehydrogenase (ubiquinone) activity	IEA Inferred from Electronic Annotation more info
ubiquinone binding	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
aerobic respiration	TAS Traceable Author Statement more info	PubMed
respiratory electron transport chain	TAS Traceable Author Statement more info
small molecule metabolic process	TAS Traceable Author Statement more info
succinate metabolic process	IEA Inferred from Electronic Annotation more info
tricarboxylic acid cycle	IEA Inferred from Electronic Annotation more info
tricarboxylic acid cycle	TAS Traceable Author Statement more info

Component	Evidence Code	Pubs
mitochondrial inner membrane	ISS Inferred from Sequence or Structural Similarity more info
mitochondrial inner membrane	TAS Traceable Author Statement more info
mitochondrial respiratory chain complex II	ISS Inferred from Sequence or Structural Similarity more info
mitochondrion	IDA Inferred from Direct Assay more info

General protein information

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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

EC 1.3.5.1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

NG_012340.1 RefSeqGene

Range

5001..40449

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_003000.2 → NP_002991.2 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor

Status: REVIEWED

Source sequence(s)

BC007840, BF796275, BU554163

Consensus CDS

CCDS176.1

UniProtKB/Swiss-Prot

P21912

Related

ENSP00000364649, OTTHUMP00000002396, ENST00000375499, OTTHUMT00000006603

Conserved Domains (2) summary

PRK05950
Location:41 – 272
Blast Score: 1124

sdhB; succinate dehydrogenase iron-sulfur subunit; Reviewed

pfam13085
Location:41 – 147
Blast Score: 367

Fer2_3; 2Fe-2S iron-sulfur cluster binding domain

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 104

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh37.p10 Primary Assembly

Genomic

NC_000001.10 Reference GRCh37.p10 Primary Assembly

Range

17345217..17380665, complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

Alternate HuRef

Genomic

AC_000133.1 Alternate HuRef

Range

15589311..15624677, complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

Alternate CHM1_1.0

Genomic

NC_018912.1 Alternate CHM1_1.0

Range

17551367..17586812, complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

Nucleotide		Protein
Heading	Accession and Version	Protein
genomic	ABBA01000262.1 (38451..55081)	None
genomic	ABBA01000263.1	None
genomic	AJ549502.2	CAE47739.1
genomic	AL049569.13 (103316..138764)	None
genomic	AMYH01000295.1 (18511..53956)	None
genomic	CH471134.1	EAW94828.1
		EAW94829.1
genomic	U17886.1	AAA80581.1
mRNA	AK312056.1	BAG34992.1
mRNA	BC007840.2	AAH07840.1
mRNA	BF796275.1	None
mRNA	BU554163.1	None
mRNA	D10245.1	BAA01089.1
mRNA	DQ403007.1	ABD77140.1
mRNA	M32246.1	AAA35708.1
mRNA	U17248.1	AAA81167.1