Acadl acyl-Coenzyme A dehydrogenase, long-chain [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Acadlprovided by MGI
Official Full Name: acyl-Coenzyme A dehydrogenase, long-chainprovided by MGI
Primary source: MGI:MGI:87866
See related: Ensembl:ENSMUSG00000026003 AllianceGenome:MGI:87866
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: LCAD
Summary: This gene encodes a homotetrameric mitochondrial flavoprotein and is a member of the acyl-CoA dehydrogenase family. Members of this family catalyze the first step of fatty acid beta-oxidation, forming a C2-C3 trans-double bond in a FAD-dependent reaction. As beta-oxidation cycles through its four steps, each member of the acyl-CoA dehydrogenase family works at an optimum fatty acid chain-length. This enzyme has its optimum length between C12- and C16-acylCoA. In mice, deficiency of this gene can cause sudden death, cardiomyopathy as well as fasting and cold intolerance. [provided by RefSeq, Nov 2012]
Expression: Broad expression in heart adult (RPKM 89.2), liver E18 (RPKM 50.6) and 22 other tissues See more
Orthologs: human all
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Genomic context

Location:: 1 C3; 1 33.64 cM

Exon count:: 11

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	1	NC_000067.7 (66869998..66902468, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	1	NC_000067.6 (66830839..66863309, complement)

Chromosome 1 - NC_000067.7 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?

The KLF7/PFKL/ACADL axis modulates cardiac metabolic remodelling during cardiac hypertrophy in male mice.
Title: The KLF7/PFKL/ACADL axis modulates cardiac metabolic remodelling during cardiac hypertrophy in male mice.
Slc22a5 haploinsufficiency does not aggravate the phenotype of the long-chain acyl-CoA dehydrogenase KO mouse.
Title: Slc22a5 haploinsufficiency does not aggravate the phenotype of the long-chain acyl-CoA dehydrogenase KO mouse.
Long-chain acyl-CoA dehydrogenase can be a source of mitochondrial hydrogen peroxide.
Title: The fatty acid oxidation enzyme long-chain acyl-CoA dehydrogenase can be a source of mitochondrial hydrogen peroxide.
LCAD-/- mice succumb to influenza from bioenergetic starvation, likely due to increased reliance upon glucose for energy.
Title: Increased mortality from influenza infection in long-chain acyl-CoA dehydrogenase knockout mice.
SIRT3 may play an essential role in attenuating lipid accumulation in the heart through the deacetylation of LCAD
Title: Mouse SIRT3 attenuates hypertrophy-related lipid accumulation in the heart through the deacetylation of LCAD.
Carnitine supplementation lowered myocardial triglycerides, normalizing myocardial triglycerides levels in LCAD Knock-out mice.
Title: Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice.
LCAD KO mice are ineffective in maintaining metabolic homeostasis during fasting, which is reflected by an impaired myocardial energy status in fasted LCAD KO mice.
Title: Myocardial energy shortage and unmet anaplerotic needs in the fasted long-chain acyl-CoA dehydrogenase knockout mouse.
the fatty acid oxidation pathway and LCAD are factors contributing to the pathophysiology of pulmonary disease
Title: Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction.
Sirtuin 3 (SIRT3) protein regulates long-chain acyl-CoA dehydrogenase by deacetylating conserved lysines near the active site.
Title: Sirtuin 3 (SIRT3) protein regulates long-chain acyl-CoA dehydrogenase by deacetylating conserved lysines near the active site.
mice with an inherited deficiency of very long-chain acyl-CoA dehydrogenase (VLCAD), were protected from high-fat diet-induced obesity and liver and muscle insulin resistance.
Title: Resistance to high-fat diet-induced obesity and insulin resistance in mice with very long-chain acyl-CoA dehydrogenase deficiency.

Submit: New GeneRIF Correction See all GeneRIFs (14)

Variation

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Alleles

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

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

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

RH136471 (e-PCR)
- UniSTS:210456

RH126755 (e-PCR)
- UniSTS:207168

AA960361 (e-PCR)
- UniSTS:158804

Acadl (e-PCR), detects polymorphism
- UniSTS:527231

ACADL (e-PCR)
- UniSTS:105159

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables fatty-acyl-CoA binding	ISO Inferred from Sequence Orthology more info
enables flavin adenine dinucleotide binding	IBA Inferred from Biological aspect of Ancestor more info
enables flavin adenine dinucleotide binding	IEA Inferred from Electronic Annotation more info
enables flavin adenine dinucleotide binding	ISO Inferred from Sequence Orthology more info
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables long-chain fatty acyl-CoA dehydrogenase activity	IBA Inferred from Biological aspect of Ancestor more info
enables long-chain fatty acyl-CoA dehydrogenase activity	IDA Inferred from Direct Assay more info	PubMed
enables long-chain fatty acyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables palmitoyl-CoA oxidase activity	IBA Inferred from Biological aspect of Ancestor more info

Process	Evidence Code	Pubs
involved_in carnitine catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in carnitine metabolic process, CoA-linked	IBA Inferred from Biological aspect of Ancestor more info
involved_in carnitine metabolic process, CoA-linked	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in fatty acid beta-oxidation using acyl-CoA dehydrogenase	IBA Inferred from Biological aspect of Ancestor more info
involved_in fatty acid beta-oxidation using acyl-CoA dehydrogenase	IDA Inferred from Direct Assay more info	PubMed
involved_in fatty acid beta-oxidation using acyl-CoA dehydrogenase	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in fatty acid beta-oxidation using acyl-CoA dehydrogenase	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within fatty acid catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in lipid catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in long-chain fatty acid catabolic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in long-chain fatty acid catabolic process	IEA Inferred from Electronic Annotation more info
involved_in long-chain fatty acid catabolic process	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of fatty acid biosynthetic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of fatty acid oxidation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of cold-induced thermogenesis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in regulation of cholesterol metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within response to cold	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in temperature homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed

Component	Evidence Code	Pubs
is_active_in cytoplasm	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
located_in mitochondrial matrix	ISO Inferred from Sequence Orthology more info
located_in mitochondrial membrane	ISO Inferred from Sequence Orthology 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	IDA Inferred from Direct Assay more info	PubMed

General protein information

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Preferred Names: long-chain specific acyl-CoA dehydrogenase, mitochondrial

Names: acetyl-Coenzyme A dehydrogenase, long-chain

NP_031407.2

EC 1.3.8.8

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_007381.4 → NP_031407.2 long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor

See identical proteins and their annotated locations for NP_031407.2

Status: REVIEWED

Source sequence(s)

AC124389, AK005140, AK151845

Consensus CDS

CCDS15023.1

UniProtKB/Swiss-Prot

B2KGC6, O35302, P51174, Q8QZR6, Q9CU29, Q9DB83

UniProtKB/TrEMBL

A0A0R4J083

Related

ENSMUSP00000027153.6, ENSMUST00000027153.6

Conserved Domains (2) summary

cd01160
Location:56 → 427

LCAD; Long chain acyl-CoA dehydrogenase

COG1960
Location:53 → 427

CaiA; Acyl-CoA dehydrogenase related to the alkylation response protein AidB [Lipid transport and metabolism]