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

Summary

Official Symbol: Acadvlprovided by MGI
Official Full Name: acyl-Coenzyme A dehydrogenase, very long chainprovided by MGI
Primary source: MGI:MGI:895149
See related: Ensembl:ENSMUSG00000018574 AllianceGenome:MGI:895149
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: vlcad
Summary: This gene encodes a homodimeric 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 C16- and C20-acylCoA and localizes to the inner mitochondrial membrane (unlike related acyl-CoA dehydrogenases). In mice, deficiency of this gene can cause ventricular arrhythmias as well as fasting and cold intolerance. [provided by RefSeq, Nov 2012]
Expression: Broad expression in heart adult (RPKM 185.6), liver E18 (RPKM 80.6) and 25 other tissues See more
Orthologs: human all
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Genomic context

Location:: 11 B3; 11 42.96 cM

Exon count:: 20

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	11	NC_000077.7 (69901009..69906254, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	11	NC_000077.6 (70010183..70015428, complement)

Chromosome 11 - NC_000077.7 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 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?

Remodeling of lipid landscape in high fat fed very-long chain acyl-CoA dehydrogenase null mice favors pro-arrhythmic polyunsaturated fatty acids and their downstream metabolites.
Title: Remodeling of lipid landscape in high fat fed very-long chain acyl-CoA dehydrogenase null mice favors pro-arrhythmic polyunsaturated fatty acids and their downstream metabolites.
Cardiac tissue citric acid cycle intermediates in exercised very long-chain acyl-CoA dehydrogenase-deficient mice fed triheptanoin or medium-chain triglyceride.
Title: Cardiac tissue citric acid cycle intermediates in exercised very long-chain acyl-CoA dehydrogenase-deficient mice fed triheptanoin or medium-chain triglyceride.
Wild Type and VLCAD-/- mice showed strong sex-dependent differences in basal metabolism and expression of proteins involved in the distinct metabolic pathways, even more prominent after treatment with octanoate.
Title: The fate of medium-chain fatty acids in very long-chain acyl‑CoA dehydrogenase deficiency (VLCADD): A matter of sex?
our results demonstrate that infection as a stress produces perturbations in metabolism in Acadvl(-/-) that differ greatly from WT infected and Acadvl(-/-) pair-fed controls. This model system will be useful for studying the effects of infection on tissue metabolism as well as evaluating interventions aimed at modulating the effects of metabolic decompensation.
Title: Tissue acylcarnitine status in a mouse model of mitochondrial β-oxidation deficiency during metabolic decompensation due to influenza virus infection.
the MCL-1 BH3 interaction with VLCAD revealed a separable, gain-of-function role for MCL-1 in the regulation of lipid metabolism.
Title: Dynamic Regulation of Long-Chain Fatty Acid Oxidation by a Noncanonical Interaction between the MCL-1 BH3 Helix and VLCAD.
Triheptanoin was not able to prevent the development of systolic dysfunction in VLCAD(-/-) mice despite an upregulation of cardiac glucose oxidation. Strikingly, the anaplerotic effects of triheptanoin were restricted to the liver.
Title: Triheptanoin: long-term effects in the very long-chain acyl-CoA dehydrogenase-deficient mouse.
observed strong upregulation of peroxisomal beta-oxidation in VLCAD(-/-) mice
Title: De novo fatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids.
SIRT3 and SIRT5 regulate the enzyme activity and cardiolipin binding of very long-chain acyl-CoA dehydrogenase
Title: SIRT3 and SIRT5 regulate the enzyme activity and cardiolipin binding of very long-chain acyl-CoA dehydrogenase.
Studies conducted with permeabilized mitochondria and different chain length acyl-CoA derivatives suggest that VLCAD is also a source of reactive oxygen species production in mitochondria of high fat diet animals.
Title: Diet-sensitive sources of reactive oxygen species in liver mitochondria: role of very long chain acyl-CoA dehydrogenases.
We demonstrate here that both dietary interventions with respect to the fat content of the diet reverse endogenous compensatory mechanisms in muscle that have evolved in VLCAD(-/-) mice resulting in pronounced energy deficiency
Title: Long-term dietary effects on substrate selection and muscle fiber type in very-long-chain acyl-CoA dehydrogenase deficient (VLCAD(-/-)) mice.

Submit: New GeneRIF Correction See all GeneRIFs (21)

Variation

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Alleles

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

Endonuclease-mediated (4)
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

Dvl2 (e-PCR), detects polymorphism
- UniSTS:142881

Acadvl (e-PCR), detects polymorphism
- UniSTS:527232

Dvl2 (e-PCR), detects polymorphism
- UniSTS:508378

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables acyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables acyl-CoA dehydrogenase activity	ISS Inferred from Sequence or Structural Similarity more info
enables fatty-acyl-CoA binding	IBA Inferred from Biological aspect of Ancestor more info
enables fatty-acyl-CoA binding	ISO Inferred from Sequence Orthology more info
enables flavin adenine dinucleotide binding	ISO Inferred from Sequence Orthology more info
enables flavin adenine dinucleotide binding	ISS Inferred from Sequence or Structural Similarity more info
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables identical protein binding	ISS Inferred from Sequence or Structural Similarity more info
enables long-chain fatty acyl-CoA dehydrogenase activity	IEA Inferred from Electronic Annotation more info
enables long-chain fatty acyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables very-long-chain fatty acyl-CoA dehydrogenase activity	IBA Inferred from Biological aspect of Ancestor more info
enables very-long-chain fatty acyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables very-long-chain fatty acyl-CoA dehydrogenase activity	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
involved_in epithelial cell differentiation	IEA Inferred from Electronic Annotation more info
involved_in fatty acid beta-oxidation	ISO Inferred from Sequence Orthology more info
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
involved_in fatty acid beta-oxidation using acyl-CoA dehydrogenase	ISS Inferred from Sequence or Structural Similarity more info
acts_upstream_of_or_within fatty acid catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
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 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
located_in mitochondrial inner membrane	IEA Inferred from Electronic Annotation more info
located_in mitochondrial inner membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrial membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrial nucleoid	IEA Inferred from Electronic Annotation more info
located_in mitochondrial nucleoid	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in nucleolus	IEA Inferred from Electronic Annotation more info
located_in nucleolus	ISO Inferred from Sequence Orthology more info
located_in nucleoplasm	IEA Inferred from Electronic Annotation more info
located_in nucleoplasm	ISO Inferred from Sequence Orthology more info

General protein information

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

Names: MVLCAD

NP_059062.1

EC 1.3.8.9

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_017366.3 → NP_059062.1 very long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor

See identical proteins and their annotated locations for NP_059062.1

Status: REVIEWED

Source sequence(s)

AI843782, AK076037, AU067294

Consensus CDS

CCDS24931.1

UniProtKB/Swiss-Prot

O35289, O55133, P50544

UniProtKB/TrEMBL

B1AR28

Related

ENSMUSP00000099634.4, ENSMUST00000102574.10

Conserved Domains (2) summary

cd01161
Location:73 → 482

VLCAD; Very long chain acyl-CoA dehydrogenase

COG1960
Location:95 → 474

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