Gene Ontology annotation through association of InterPro records with GO terms
Manual transfer of experimentally-verified manual GO annotation data to orthologs by curator judgment of sequence similarity
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt
Gene Ontology annotation based on curation of immunofluorescence data
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Automatic Gene Ontology annotation based on Rhea mapping
Electronic Gene Ontology annotations created by ARBA machine learning models
Combined Automated Annotation using Multiple IEA Methods
Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids.
The layered structure of human mitochondrial DNA nucleoids.
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Table 1 assigns ACADVL/NP_000009 to Class I, with independent peptide counts of 3 for native anti-TFAM, 0 for native anti-mtSSB, 3 for cross-linked preparation 1 and 0 for preparation 2. This supports biochemical nucleoid association in one preparation of each category, without establishing direct DNA binding or genome maintenance.
Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase.
Identification and characterization of new long chain acyl-CoA dehydrogenases.
Analysis of proteomic changes induced upon cellular differentiation of the human intestinal cell line Caco-2.
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Table 1 reports a 3.25-fold increase in ACADVL during Caco-2 differentiation; the study analyzes expression-associated metabolic changes.
"proteins involved in xenobiotic and drug metabolism as well as in lipid metabolism were upregulated upon cellular differentiation"
A reference map of the human binary protein interactome.
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
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Defines a high-confidence human mitochondrial proteome (MitoCoP) of 1,134 protein-coding genes. The ACADVL-specific supplementary identification was not independently re-extracted; its curated mitochondrial assignment agrees with targeted VLCAD localization studies.
"We defined a human mitochondrial high-confidence proteome (MitoCoP) of 1,134 protein-coding genes"
Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood.
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VLCAD catalyzes the first step of beta-oxidation of long-chain fatty acids, the major energy source in heart; VLCAD deficiency reduces myocardial beta-oxidation and energy production and causes cardiomyopathy and sudden death.
"VLCAD deficiency reduces myocardial fatty acid beta-oxidation and energy production and is associated with cardiomyopathy and sudden death in childhood"
Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients.
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Original cloning of human VLCAD (655 aa precursor; 40-aa leader peptide; 615-aa mature protein). Demonstrates that VLCAD deficiency impairs long-chain (palmitate) beta-oxidation and that restoring ~20% VLCAD activity restores palmitate beta-oxidation flux.
"raising VLCAD activity to approximately 20% of normal control fibroblast activity raised palmitic acid beta-oxidation flux to the level found in control fibroblasts"
A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase.
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First identification of VLCAD deficiency as a distinct disease, based on very low palmitoyl-CoA (C16) dehydrogenase activity and loss of anti-VLCAD immunoreactivity in patients.
"found to have a novel disease, VLCAD deficiency, as judged from the results of very low palmitoyl-CoA dehydrogenase activity and the lack of immunoreactivity toward antibody raised to purified VLCAD"
Catalytic and FAD-binding residues of mitochondrial very long chain acyl-coenzyme A dehydrogenase.
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VLCAD is one of four flavoproteins catalyzing the initial step of mitochondrial beta-oxidation. Glu-422 is the catalytic base (E422Q abolishes activity by preventing the VLCAD-palmitoyl-CoA charge-transfer complex); Phe-418 is required for FAD binding/reduction and FAD loss destabilizes folding.
"Glu-422 of VLCAD has been presumed to be the catalytic residue that abstracts the alpha-proton in the alphabeta-dehydrogenation reaction. Replacing Glu-422 with glutamine (E422Q) caused a loss of enzyme activity"
Very-long-chain acyl-CoA dehydrogenase subunit assembles to the dimer form on mitochondrial inner membrane.
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Mature VLCAD is a homodimer of ~70 kDa subunits associated with the mitochondrial inner membrane; membrane association precedes and is required for dimer assembly. The monomeric mutant S583W fails to bind the membrane and remains soluble in the matrix.
"association of VLCAD protein with mitochondrial inner membrane is necessary for dimer assembly and formation of mature VLCAD"
palmitoyl-CoA+FAD => trans-Hexadec-2-enoyl-CoA+FADH2
Differential induction of genes in liver and brown adipose tissue regulated by peroxisome proliferator-activated receptor-alpha during fasting and cold exposure in acyl-CoA dehydrogenase-deficient mice.
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Both LCAD- and VLCAD-deficient mice fail to maintain temperature during cold exposure. The abstract also reports tissue-specific compensatory expression changes, without resolving every transferred regulatory assertion.
"neither mouse model can maintain core body temperature when exposed to cold"
UniProt record for human ACADVL (P49748)
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The curated catalytic record assigns tetracosanoyl-CoA dehydrogenation RHEA:47232 with experimental evidence attributed to PMID:21237683.
"Reaction=tetracosanoyl-CoA + oxidized [electron-transfer flavoprotein]
CC + H(+) = (2E)-tetracosenoyl-CoA + reduced [electron-transfer
CC flavoprotein]; Xref=Rhea:RHEA:47232, Rhea:RHEA-COMP:10685, Rhea:RHEA-
CC COMP:10686, ChEBI:CHEBI:15378, ChEBI:CHEBI:57692, ChEBI:CHEBI:58307,
CC ChEBI:CHEBI:65052, ChEBI:CHEBI:74693;
CC Evidence={ECO:0000269|PubMed:21237683};"