Sdhb succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Sdhbprovided by MGI
Official Full Name: succinate dehydrogenase complex, subunit B, iron sulfur (Ip)provided by MGI
Primary source: MGI:MGI:1914930
See related: Ensembl:ENSMUSG00000009863 AllianceGenome:MGI:1914930
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: 0710008N11Rik
Summary: Predicted to enable succinate dehydrogenase (ubiquinone) activity. Predicted to be involved in aerobic respiration; respiratory electron transport chain; and succinate metabolic process. Predicted to act upstream of or within tricarboxylic acid cycle. Located in mitochondrion. Part of mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone). Is expressed in several structures, including alimentary system; brain; genitourinary system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Carney-Stratakis syndrome; gastrointestinal stromal tumor; mitochondrial complex II deficiency; paraganglioma; and pheochromocytoma. Orthologous to human SDHB (succinate dehydrogenase complex iron sulfur subunit B). [provided by Alliance of Genome Resources, Apr 2022]
Expression: Ubiquitous expression in heart adult (RPKM 390.1), kidney adult (RPKM 243.7) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 4 D3; 4 73.24 cM

Exon count:: 8

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	4	NC_000070.7 (140688582..140706509)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	4	NC_000070.6 (140961271..140979198)

Chromosome 4 - NC_000070.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Loss of SDHB Promotes Dysregulated Iron Homeostasis, Oxidative Stress, and Sensitivity to Ascorbate.
Title: Loss of SDHB Promotes Dysregulated Iron Homeostasis, Oxidative Stress, and Sensitivity to Ascorbate.
GIPC2 is an endocrine-specific tumor suppressor gene for both sporadic and hereditary tumors of RET- and SDHB-, but not VHL-associated clusters of pheochromocytoma/paraganglioma.
Title: GIPC2 is an endocrine-specific tumor suppressor gene for both sporadic and hereditary tumors of RET- and SDHB-, but not VHL-associated clusters of pheochromocytoma/paraganglioma.
TET-Mediated Hypermethylation Primes SDH-Deficient Cells for HIF2alpha-Driven Mesenchymal Transition.
Title: TET-Mediated Hypermethylation Primes SDH-Deficient Cells for HIF2α-Driven Mesenchymal Transition.
the present study identified a potential role for the tumor microenvironment in influencing phenotypic features of SDHB-mutated Pheochromocytomas and paragangliomas.
Title: Primary fibroblast co-culture stimulates growth and metabolism in Sdhb-impaired mouse pheochromocytoma MTT cells.
Data show that lack of succinate dehydrogenase (SDH) activity commits cells to consume extracellular pyruvate.
Title: Pyruvate carboxylation enables growth of SDH-deficient cells by supporting aspartate biosynthesis.
Using Sdhb(+/-) mice, we provide evidence that pituitary hyperplasia in SDHx-deficient cells may be the initial abnormality in the cascade of events leading to pituitary adenoma formation.
Title: Pituitary adenoma with paraganglioma/pheochromocytoma (3PAs) and succinate dehydrogenase defects in humans and mice.
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.
Data show that two subunits of complex II (succinate dehydrogenase, or SDH), SDHA and SDHB, interacted specifically with SIRT3.
Title: Succinate dehydrogenase is a direct target of sirtuin 3 deacetylase activity.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Gene trapped (1)
Targeted (6) 1 citation
Endonuclease-mediated (3)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

NoName (e-PCR)
- UniSTS:235080

Sdhb (e-PCR), detects polymorphism
- UniSTS:507209

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables 2 iron, 2 sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables 3 iron, 4 sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables 4 iron, 4 sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables electron transfer activity	IEA Inferred from Electronic Annotation more info
enables iron-sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables oxidoreductase activity	IEA Inferred from Electronic Annotation more info
enables succinate dehydrogenase (quinone) activity	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in aerobic respiration	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrial electron transport, succinate to ubiquinone	NAS Non-traceable Author Statement more info	PubMed
involved_in proton motive force-driven mitochondrial ATP synthesis	NAS Non-traceable Author Statement more info	PubMed
involved_in respiratory electron transport chain	IBA Inferred from Biological aspect of Ancestor more info
involved_in respiratory electron transport chain	ISO Inferred from Sequence Orthology more info
involved_in succinate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in tricarboxylic acid cycle	NAS Non-traceable Author Statement more info	PubMed

Component	Evidence Code	Pubs
located_in membrane	IEA Inferred from Electronic Annotation more info
located_in mitochondrial inner membrane	HDA more info	PubMed
located_in mitochondrial inner membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrial inner membrane	NAS Non-traceable Author Statement more info	PubMed
is_active_in mitochondrial membrane	IBA Inferred from Biological aspect of Ancestor more info
part_of mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)	IDA Inferred from Direct Assay more info	PubMed
part_of mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)	NAS Non-traceable Author Statement more info	PubMed
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in nucleoplasm	ISO Inferred from Sequence Orthology more info
located_in plasma membrane	ISO Inferred from Sequence Orthology more info
part_of respiratory chain complex II	IDA Inferred from Direct Assay more info	PubMed
part_of succinate dehydrogenase complex (ubiquinone)	IDA Inferred from Direct Assay more info	PubMed

General protein information

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Preferred Names: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial

Names: ip; iron-sulfur subunit of complex II; succinate dehydrogenase complex subunit B

NP_001342444.1

EC 1.3.5.1

NP_075863.2

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.

mRNA and Protein(s)

NM_001355515.1 → NP_001342444.1 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform 2

Status: VALIDATED

Description

Transcript Variant: This variant (2) uses an alternate in-frame splice site in the 5' coding region, compared to variant 1. The encoded isoform (2) has the same N- and C-termini, but is two aa shorter than isoform 1.

Source sequence(s)

AI835380, BC051934, BY111239

UniProtKB/TrEMBL

Q3TE45

Conserved Domains (1) summary

PLN00129
Location:1 → 271

PLN00129; succinate dehydrogenase [ubiquinone] iron-sulfur subunit
NM_023374.4 → NP_075863.2 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform 1 precursor

See identical proteins and their annotated locations for NP_075863.2

Status: VALIDATED

Description

Transcript Variant: This variant (1) represents the longer transcript and encodes the longer isoform (1).

Source sequence(s)

AI835380, BC051934

Consensus CDS

CCDS18858.1

UniProtKB/Swiss-Prot

Q3TF82, Q9CQA3, Q9DC91

UniProtKB/TrEMBL

Q3TE45

Related

ENSMUSP00000010007.9, ENSMUST00000010007.9

Conserved Domains (3) summary

PRK05950
Location:43 → 274

sdhB; succinate dehydrogenase iron-sulfur subunit; Reviewed

pfam13085
Location:43 → 149

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

pfam13534
Location:187 → 260

Fer4_17; 4Fe-4S dicluster domain