Sdhd succinate dehydrogenase complex, subunit D, integral membrane protein [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Sdhdprovided by MGI
Official Full Name: succinate dehydrogenase complex, subunit D, integral membrane proteinprovided by MGI
Primary source: MGI:MGI:1914175
See related: Ensembl:ENSMUSG00000000171 AllianceGenome:MGI:1914175
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: QPs3; cybS; CII-4; AVLL5809; PRO19626; 3110001M13Rik
Summary: Enables succinate dehydrogenase (quinone) activity. Acts upstream of or within cellular response to hypoxia and regulation of catecholamine secretion. Located in mitochondrion. Part of respiratory chain complex II (succinate dehydrogenase). Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Carney-Stratakis syndrome; mitochondrial complex II deficiency; and paraganglioma. Orthologous to human SDHD (succinate dehydrogenase complex subunit D). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in heart adult (RPKM 247.8), duodenum adult (RPKM 208.9) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 9 A5.3; 9 27.75 cM

Exon count:: 4

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	9	NC_000075.7 (50507640..50515149, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	9	NC_000075.6 (50596340..50603849, complement)

Chromosome 9 - NC_000075.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Breast cancer-associated macrophages promote tumorigenesis by suppressing succinate dehydrogenase in tumor cells.
Title: Breast cancer-associated macrophages promote tumorigenesis by suppressing succinate dehydrogenase in tumor cells.
Sdhd knockout from the thyroid is sufficient to cause excess thyroid cell growth in mice, which may play a role in tumor initiation
Title: Sdhd ablation promotes thyroid tumorigenesis by inducing a stem-like phenotype.
the actual dependency of undifferentiated hematopoietic stem cells on mitochondrial function, we have performed an analysis of the hematopoiesis in a mouse mutant, named SDHD-ESR, with inducible deletion of the mitochondrial protein-encoding SdhD gene, is reported.
Title: Sensitivity of hematopoietic stem cells to mitochondrial dysfunction by SdhD gene deletion.
Loss of SDH activity leads to changes in the metabolism of non-essential amino acids.
Title: Loss of succinate dehydrogenase activity results in dependency on pyruvate carboxylation for cellular anabolism.
In mice with conditional knockout of Sdhd, we found that the Cdkn1a gene was up-regulated in kidney and adrenal. This gene encodes the cyclin-dependent kinase inhibitor p21(WAF1/Cip1), a factor implicated in cell cycle, senescence, and cancer.
Title: A conditional mouse mutant in the tumor suppressor SdhD gene unveils a link between p21(WAF1/Cip1) induction and mitochondrial dysfunction.
complete loss of SdhD is not sufficient to induce tumorigenesis in mice
Title: Differential impairment of catecholaminergic cell maturation and survival by genetic mitochondrial complex II dysfunction.
SDHD is required for the formation of a stable mitochondrial complex II, and it is selectively important for hypoxic pulmonary vasoconstriction of intra-acinar vessels.
Title: Mitochondrial complex II is essential for hypoxia-induced pulmonary vasoconstriction of intra- but not of pre-acinar arteries.
Knockout of Sdhd in the mouse does not result in a disease phenotype; H19 may not be an initiator of PGL/PC tumorigenesis
Title: Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma.
Mitochondrial Sdhd is required for early embryogenesis, and its partial deficiency results in persistent carotid body glomus cell activation with full responsiveness to cell hypoxia.
Title: The mitochondrial SDHD gene is required for early embryogenesis, and its partial deficiency results in persistent carotid body glomus cell activation with full responsiveness to hypoxia.

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Variation

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Alleles

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

Endonuclease-mediated (1)
Targeted (3) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

RH134598 (e-PCR)
- UniSTS:217878

RH126393 (e-PCR)
- UniSTS:163101

Sdhd (e-PCR), detects polymorphism
- UniSTS:507211

RH127747 (e-PCR)
- UniSTS:211057

RH135849 (e-PCR)
- UniSTS:209847

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables heme binding	IBA Inferred from Biological aspect of Ancestor more info
enables heme binding	ISS Inferred from Sequence or Structural Similarity more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables succinate dehydrogenase (quinone) activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables ubiquinone binding	IBA Inferred from Biological aspect of Ancestor more info
enables ubiquinone binding	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within cellular response to hypoxia	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in mitochondrial electron transport, succinate to ubiquinone	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
acts_upstream_of_or_within regulation of catecholamine secretion	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in tricarboxylic acid cycle	IBA Inferred from Biological aspect of Ancestor more info
involved_in tricarboxylic acid cycle	IEA Inferred from Electronic Annotation more info
involved_in tricarboxylic acid cycle	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 mitochondrial inner membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrial inner membrane	ISS Inferred from Sequence or Structural Similarity more info
located_in mitochondrial inner membrane	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
is_active_in mitochondrion	IMP Inferred from Mutant Phenotype more info	PubMed
located_in mitochondrion	IMP Inferred from Mutant Phenotype more info	PubMed
part_of respiratory chain complex II (succinate dehydrogenase)	IBA Inferred from Biological aspect of Ancestor more info
part_of respiratory chain complex II (succinate dehydrogenase)	IDA Inferred from Direct Assay more info	PubMed
part_of respiratory chain complex II (succinate dehydrogenase)	ISS Inferred from Sequence or Structural Similarity more info

General protein information

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Preferred Names: succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial

Names: malate dehydrogenase [quinone] cytochrome b small subunit; succinate dehydrogenase complex subunit D; succinate-ubiquinone oxidoreductase cytochrome b small subunit; succinate-ubiquinone reductase membrane anchor subunit

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_025848.3 → NP_080124.1 succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial

See identical proteins and their annotated locations for NP_080124.1

Status: VALIDATED

Source sequence(s)

AC025500, AK135970, BF662071

Consensus CDS

CCDS40623.1

UniProtKB/Swiss-Prot

A6H629, Q3UX11, Q9CXV1

UniProtKB/TrEMBL

Q9D6J9

Related

ENSMUSP00000000175.5, ENSMUST00000000175.6

Conserved Domains (1) summary

cd03496
Location:60 → 158

SQR_TypeC_CybS; SQR catalyzes the oxidation of succinate to fumarate coupled to the reduction of quinone to quinol. Eukaryotic SQRs reduce high potential quinones such as ubiquinone. SQR is also called succinate dehydrogenase or Complex II, and is part of the citric ...