Dlst dihydrolipoamide S-succinyltransferase [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Dlstprovided by MGI
Official Full Name: dihydrolipoamide S-succinyltransferaseprovided by MGI
Primary source: MGI:MGI:1926170
See related: Ensembl:ENSMUSG00000004789 AllianceGenome:MGI:1926170
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: DLTS; 1600017E01Rik; 4632413C10Rik; 4930529O08Rik
Summary: Predicted to enable dihydrolipoyllysine-residue succinyltransferase activity; heat shock protein binding activity; and protein-folding chaperone binding activity. Predicted to be involved in 2-oxoglutarate metabolic process; nucleoside phosphate metabolic process; and tricarboxylic acid cycle. Located in mitochondrion and myelin sheath. Part of oxoglutarate dehydrogenase complex. Is expressed in several structures, including alimentary system; metanephros; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Alzheimer's disease and paraganglioma. Orthologous to human DLST (dihydrolipoamide S-succinyltransferase). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in heart adult (RPKM 165.8), kidney adult (RPKM 107.4) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 12 D1; 12 39.55 cM

Exon count:: 15

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	12	NC_000078.7 (85157597..85180865)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	12	NC_000078.6 (85110822..85134091)

Chromosome 12 - NC_000078.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

DLST protein levels were decreased on pressure overload in wild-type mice, but levels were maintained in microRNA-146a knock-out mice.
Title: Inhibition of MicroRNA-146a and Overexpression of Its Target Dihydrolipoyl Succinyltransferase Protect Against Pressure Overload-Induced Cardiac Hypertrophy and Dysfunction.
ATP consumption is demonstrated in respiration-impaired isolated and in situ neuronal somal mitochondria from transgenic mice with a deficiency of dihydrolipoyl dehydrogenase.
Title: The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation.
These results imply that diminished KGDHC activity has the potential to induce the reduction in glucose utilization that is seen in several neurodegenerative diseases.
Title: Brain [U-13 C]glucose metabolism in mice with decreased α-ketoglutarate dehydrogenase complex activity.
A reduction in the DLST subunit of alpha-ketoglutarate dehydrogenase complex (KGDHC) alters the overall KGDHC activity and susceptibility to mitochondrial toxins and may contribute to the pathogenesis of neurodegenerative diseases.
Title: Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins.

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Variation

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Alleles

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

Endonuclease-mediated (1)
Gene trapped (2) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

MARC_2710-2711:991933212:3 (e-PCR)
- UniSTS:230938

NoName (e-PCR)
- UniSTS:236906

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables acyltransferase activity	ISO Inferred from Sequence Orthology more info
enables acyltransferase activity	ISS Inferred from Sequence or Structural Similarity more info
enables dihydrolipoyllysine-residue succinyltransferase activity	IBA Inferred from Biological aspect of Ancestor more info
enables dihydrolipoyllysine-residue succinyltransferase activity	ISO Inferred from Sequence Orthology more info
enables dihydrolipoyllysine-residue succinyltransferase activity	ISS Inferred from Sequence or Structural Similarity more info
enables heat shock protein binding	ISO Inferred from Sequence Orthology more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein-folding chaperone binding	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in 2-oxoglutarate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in 2-oxoglutarate metabolic process	ISS Inferred from Sequence or Structural Similarity more info
involved_in L-lysine catabolic process to acetyl-CoA via saccharopine	IEA Inferred from Electronic Annotation more info
involved_in NADH metabolic process	ISO Inferred from Sequence Orthology more info
involved_in succinyl-CoA metabolic process	ISS Inferred from Sequence or Structural Similarity more info
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	ISS Inferred from Sequence or Structural Similarity more info

Component	Evidence Code	Pubs
located_in cytosol	IEA Inferred from Electronic Annotation more info
located_in cytosol	ISO Inferred from Sequence Orthology more info
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in myelin sheath	HDA more info	PubMed
located_in nucleoplasm	IEA Inferred from Electronic Annotation more info
located_in nucleoplasm	ISO Inferred from Sequence Orthology more info
located_in nucleus	ISO Inferred from Sequence Orthology more info
located_in nucleus	ISS Inferred from Sequence or Structural Similarity more info
part_of oxoadipate dehydrogenase complex	IEA Inferred from Electronic Annotation more info
part_of oxoglutarate dehydrogenase complex	IMP Inferred from Mutant Phenotype more info	PubMed
part_of oxoglutarate dehydrogenase complex	ISO Inferred from Sequence Orthology more info
part_of oxoglutarate dehydrogenase complex	ISS Inferred from Sequence or Structural Similarity more info

General protein information

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Preferred Names: dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial

Names: 2-oxoglutarate dehydrogenase complex component E2; E2K; OGDC-E2; dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex); dihydrolipoamide succinyltransferase component of 2-oxoglutarate dehydrogenase complex

NP_084501.1

EC 2.3.1.61

XP_006516455.1

EC 2.3.1.61

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_030225.4 → NP_084501.1 dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial

See identical proteins and their annotated locations for NP_084501.1

Status: VALIDATED

Source sequence(s)

AK158877, AK168570, CJ308553

Consensus CDS

CCDS26052.1

UniProtKB/Swiss-Prot

Q3UEA0, Q4FK55, Q8CIE8, Q9D2G2

Related

ENSMUSP00000060346.9, ENSMUST00000053811.10

Conserved Domains (3) summary

cd06849
Location:72 → 144

lipoyl_domain; Lipoyl domain of the dihydrolipoyl acyltransferase component (E2) of 2-oxo acid dehydrogenases. 2-oxo acid dehydrogenase multienzyme complexes, like pyruvate dehydrogenase (PDH), 2-oxoglutarate dehydrogenase (OGDH) and branched-chain 2-oxo acid ...

TIGR01347
Location:72 → 454

sucB; 2-oxoglutarate dehydrogenase complex dihydrolipoamide succinyltransferase (E2 component)

pfam00198
Location:239 → 452

2-oxoacid_dh; 2-oxoacid dehydrogenases acyltransferase (catalytic domain)

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

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

Reference GRCm39 C57BL/6J

Genomic

NC_000078.7 Reference GRCm39 C57BL/6J

Range

85157597..85180865

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_006516392.5 → XP_006516455.1 dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial isoform X1

Conserved Domains (1) summary

TIGR01347
Location:72 → 453

sucB; 2-oxoglutarate dehydrogenase complex dihydrolipoamide succinyltransferase (E2 component)