{ "uniprot_canonical_ac": "Q60759-1", "uniprot_ac": "Q60759", "uniprot_id": "GCDH_MOUSE", "mass": { "chemical_mass": 48606.0, "monoisotopic_mass": 48606.0 }, "refseq": { "ac": "NP_032123", "name": "glutaryl-CoA dehydrogenase, mitochondrial", "summary": "", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123" }, "gene_names": [ { "name": "Gcdh", "resource": "UniProtKB", "type": "recommended", "url": "https://www.uniprot.org/uniprot/Q60759", "id": "Q60759" }, { "name": "9030411L18; GCD", "resource": "RefSeq", "type": "synonym", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123", "id": "NP_032123" }, { "name": "Gcdh", "resource": "RefSeq", "type": "synonym", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123", "id": "NP_032123" } ], "protein_names": [ { "name": "Glutaryl-CoA dehydrogenase, mitochondrial", "resource": "UniProtKB", "type": "recommended", "url": "", "id": "Q60759" }, { "name": "1.3.8.6", "resource": "UniProtKB", "type": "synonym", "url": "", "id": "Q60759" }, { "name": "GCD", "resource": "UniProtKB", "type": "synonym", "url": "", "id": "Q60759" }, { "name": "glutaryl-CoA dehydrogenase, mitochondrial", "resource": "RefSeq", "type": "synonym", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123", "id": "NP_032123" } ], "function": [ { "annotation": "Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor.", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759#function" } ] }, { "annotation": "Deletion of 2-aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1.", "evidence": [ { "database": "RefSeq", "id": "NP_032123", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123" }, { "database": "PubMed", "id": "32567100", "url": "https://glygen.org/publication/PubMed/32567100" } ] }, { "annotation": "Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum-Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate.", "evidence": [ { "database": "RefSeq", "id": "NP_032123", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123" }, { "database": "PubMed", "id": "35639256", "url": "https://glygen.org/publication/PubMed/35639256" } ] } ], "disease": [ { "disease_id": "DOID:0111254", "recommended_name": { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "glutaric acidemia I", "description": "An organic acidemia characterized by impaired lysine, hydroxylysine, and tryptophan metabolism, increased urinary excretion of glutaric acid, and accumulation of 3-hydroxyglutaric and glutaric acid, resulting in striatal injury and a severe dystonic dyskinetic movement disorder that has_material_basis_in homozygous or compound heterozygous mutation in the GCDH gene on chromosome 19p13." }, "synonyms": [ { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "GA1", "description": "" }, { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "glutaric academia type 1", "description": "" }, { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "glutaric aciduria 1", "description": "" }, { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "glutaric aciduria type I", "description": "" }, { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "glutaryl-coA dehydrogenase deficiency", "description": "" }, { "id": "DOID:0111254", "resource": "Disease Ontology", "url": "http://disease-ontology.org/term/DOID:0111254", "name": "glutaryl-coenzyme A dehydrogenase deficiency", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaryl-CoA dehydrogenase deficiency", "description": "Glutaryl-CoA dehydrogenase (GCDH) deficiency (GDD) is an autosomal recessive neurometabolic disorder clinically characterized by encephalopathic crises resulting in striatal injury and a severe dystonic dyskinetic movement disorder." }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "GA1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "GCDHD", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric acidemia type 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric aciduria type 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric aciduria, type 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaricaciduria, type I", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaryl-CoA dehydrogenase deficiency", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaryl-coenzyme A dehydrogenase deficiency", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "Ga 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric acidemia 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric acidemia I", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric acidemia type I", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric acidemia, type 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric aciduria 1", "description": "" }, { "id": "MONDO:0009281", "resource": "MONDO", "url": "https://monarchinitiative.org/disease/MONDO:0009281", "name": "glutaric aciduria type I", "description": "" }, { "id": "MIM:231670", "resource": "MIM", "url": "https://www.omim.org/entry/231670", "name": "Glutaric aciduria 1", "description": "Glutaric aciduria 1" } ], "evidence": [ { "id": "0111254", "database": "Alliance of Genome Resources", "url": "https://www.alliancegenome.org/disease/DOID:0111254" } ] } ], "snv": [], "mutagenesis": [], "expression_tissue": [], "expression_disease": [], "species": [ { "name": "Mus musculus", "taxid": 10090, "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "common_name": "Mouse" } ], "gene": [ { "name": "Gcdh", "url": "https://www.uniprot.org/uniprot/Q60759", "locus": { "chromosome": "8", "start_pos": 85613022, "end_pos": 85620550, "strand": "+", "evidence": [ { "database": "Ensembl Gene", "id": "ENSMUSG00000003809", "url": "https://www.ensembl.org/id/ENSMUSG00000003809" } ] } } ], "crossref": [ { "id": "ENSMUSG00000003809", "url": "https://bgee.org/?page=gene&gene_id=ENSMUSG00000003809", "database": "Bgee", "categories": [ "Gene Expression" ] }, { "id": "1.3.8.6", "url": "https://www.brenda-enzymes.org/enzyme.php?ecno=1.3.8.6&UniProtAcc=Q60759", "database": "BRENDA", "categories": [ "Enzyme and Pathway" ] }, { "id": "cd01151", "url": "https://www.ncbi.nlm.nih.gov/Structure/cdd/cddsrv.cgi?uid=cd01151", "database": "CDD", "categories": [ "Protein Classification" ] }, { "id": "270076", "url": "https://www.ncbi.nlm.nih.gov/gene/270076", "database": "GeneID", "categories": [ "Genome Annotation" ] }, { "id": "Q60759", "url": "https://www.ebi.ac.uk/intact/interactors/id:Q60759*", "database": "IntAct", "categories": [ "Other" ] }, { "id": "IPR006089", "url": "https://www.ebi.ac.uk/interpro/entry/IPR006089", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR006091", "url": "https://www.ebi.ac.uk/interpro/entry/IPR006091", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR009075", "url": "https://www.ebi.ac.uk/interpro/entry/IPR009075", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR009100", "url": "https://www.ebi.ac.uk/interpro/entry/IPR009100", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR013786", "url": "https://www.ebi.ac.uk/interpro/entry/IPR013786", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR036250", "url": "https://www.ebi.ac.uk/interpro/entry/IPR036250", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR037069", "url": "https://www.ebi.ac.uk/interpro/entry/IPR037069", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR046373", "url": "https://www.ebi.ac.uk/interpro/entry/IPR046373", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "mmu:270076", "url": "https://www.genome.jp/dbget-bin/get_linkdb?-t+pathway+mmu:270076", "database": "KEGG Pathway", "categories": [ "Enzyme and Pathway" ] }, { "id": "104541", "url": "http://www.informatics.jax.org/marker/MGI:104541", "database": "MGI", "categories": [ "Organism-specific" ] }, { "id": "AAMDCYD", "url": "https://omabrowser.org/oma/group/AAMDCYD/", "database": "OMA", "categories": [ "Protein Classification" ] }, { "id": "275353at2759", "url": "http://www.orthodb.org/?query=275353at2759", "database": "OrthoDB", "categories": [ "Protein Classification" ] }, { "id": "PTHR42807", "url": "http://www.pantherdb.org/panther/family.do?clsAccession=PTHR42807", "database": "PANTHER", "categories": [ "Protein Classification" ] }, { "id": "PTHR42807:SF1", "url": "http://www.pantherdb.org/panther/family.do?clsAccession=PTHR42807:SF1", "database": "PANTHER", "categories": [ "Protein Classification" ] }, { "id": "PF00441", "url": "http://pfam.xfam.org/family/PF00441", "database": "Pfam", "categories": [ "Protein Classification" ] }, { "id": "PF02770", "url": "http://pfam.xfam.org/family/PF02770", "database": "Pfam", "categories": [ "Protein Classification" ] }, { "id": "PF02771", "url": "http://pfam.xfam.org/family/PF02771", "database": "Pfam", "categories": [ "Protein Classification" ] }, { "id": "PR_Q60759", "url": "http://purl.obolibrary.org/obo/PR_Q60759", "database": "PRO", "categories": [ "Protein" ] }, { "id": "R-MMU-71064", "url": "https://www.reactome.org/PathwayBrowser/#R-MMU-71064", "database": "Reactome", "categories": [ "Protein Classification" ] }, { "id": "NP_032123", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123", "database": "RefSeq", "categories": [ "Protein" ] }, { "id": "13389", "url": "https://www.rhea-db.org/rhea/13389", "database": "Rhea", "categories": [ "Enzyme and Pathway" ] }, { "id": "A0A0A0MQ68", "url": "https://www.uniprot.org/uniprot/A0A0A0MQ68", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "A0A0A0MQ68-1", "url": "https://www.uniprot.org/uniprot/A0A0A0MQ68-1", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "D3Z4I2", "url": "https://www.uniprot.org/uniprot/D3Z4I2", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "D3Z4I2-1", "url": "https://www.uniprot.org/uniprot/D3Z4I2-1", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "Q60759-1", "url": "https://www.uniprot.org/uniprot/Q60759-1", "database": "UniProtKB", "categories": [ "Protein" ] } ], "sequence": { "sequence": "MSLRGVSARLLSRRSGLRFPRFPRTWSSAAAHTEKTQIRPAKSSRPVFDWKDPLILEEQLTADEKLIRDTFRNYCQERLMSRILLANRNEVFHRDIVYEMGELGVLGPTIKGYGCAGVSSVAYGLLTRELERVDSGYRSMMSVQSSLVMHPIYTYGSEEQRQKYLPGLAKGELLGCFGLTEPNHGSDPGGMETRARHNPSNQSYTLSGTKTWITNSPVADLFIVWARCEDNCIRGFILEKGMRGLSAPRIEGKFSLRASATGMIIMDSVEVPEENVLPNVSSLAGPFGCLNTARYGITWGVLGAAEFCLHTARQYALDRIQFGVPLARNQLVQKKLADMLTEITLGLHACLQLGRLKDQDKATPEMVSMLKRNNCGKALDIARQARDILGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTVGK", "length": 438, "header": "sp|Q60759-1|GCDH_MOUSE Isoform 1 of Glutaryl-CoA dehydrogenase, mitochondrial OS=Mus musculus OX=10090 GN=Gcdh PE=1 SV=2 CANONICAL=Q60759-1" }, "isoforms": [ { "isoform_ac": "A0A0A0MQ68-1", "url": "", "sequence": { "sequence": "MVPAMLTGYMSLRGVSARLLSRRSGLRFPRFPRTWSSAAAHTEKTQIRPAKSSRPVFDWKDPLILEEQLTADEKLIRDTFRNYCQERLMSRILLANRNEVFHRDIVYEMGELGVLGPTIKGYGCAGVSSVAYGLLTRELERVDSGYRSMMSVQSSLVMHPIYTYGSEEQRQKYLPGLAKGELLGCFGLTEPNHGSDPGGMETRARHNPSNQSYTLSGTKTWITNSPVADLFIVWARCEDNCIRGFILEKGMRGLSAPRIEGKFSLRASATGMIIMDSVEVPEENVLPNVSSLAGPFGCLNTARYGITWGVLGAAEFCLHTARQYALDRIQFGVPLARNQLVQKKLADMLTEITLGLHACLQLGRLKDQDKATPEMVSMLKRNNCGKALDIARQARDILGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTVGK", "length": 447, "header": "tr|A0A0A0MQ68-1|GCDH_MOUSE Computationally mapped isoform of Glutaryl-CoA dehydrogenase, mitochondrial OS=Mus musculus OX=10090 GN=Gcdh PE=1 SV=1 CANONICAL=Q60759-1" }, "locus": { "chromosome": "8", "start_pos": 85620310, "end_pos": 85613612, "strand": "+", "evidence": [ { "database": "Ensembl Transcript", "id": "ENSMUST00000003907.14", "url": "https://www.ensembl.org/id/ENSMUST00000003907.14" }, { "database": "Ensembl Peptide", "id": "ENSMUSP00000003907.9", "url": "https://www.ensembl.org/id/ENSMUSP00000003907.9" } ] } }, { "isoform_ac": "D3Z4I2-1", "url": "", "sequence": { "sequence": "MVPAMLTGYMSLRGVSARLLSRRSGLRFPRFPRTWSSAAAHTEKTQIRPAKSSRPVFDWKDPLILEEQLTADEKL", "length": 75, "header": "tr|D3Z4I2-1|GCDH_MOUSE Computationally mapped isoform of Glutaryl-CoA dehydrogenase, mitochondrial OS=Mus musculus OX=10090 GN=Gcdh PE=1 SV=2 CANONICAL=Q60759-1" }, "locus": { "chromosome": "8", "start_pos": 85620310, "end_pos": 85619723, "strand": "+", "evidence": [ { "database": "Ensembl Transcript", "id": "ENSMUST00000142748.2", "url": "https://www.ensembl.org/id/ENSMUST00000142748.2" }, { "database": "Ensembl Peptide", "id": "ENSMUSP00000116584.3", "url": "https://www.ensembl.org/id/ENSMUSP00000116584.3" } ] } }, { "isoform_ac": "Q60759-1", "url": "", "sequence": { "sequence": "MSLRGVSARLLSRRSGLRFPRFPRTWSSAAAHTEKTQIRPAKSSRPVFDWKDPLILEEQLTADEKLIRDTFRNYCQERLMSRILLANRNEVFHRDIVYEMGELGVLGPTIKGYGCAGVSSVAYGLLTRELERVDSGYRSMMSVQSSLVMHPIYTYGSEEQRQKYLPGLAKGELLGCFGLTEPNHGSDPGGMETRARHNPSNQSYTLSGTKTWITNSPVADLFIVWARCEDNCIRGFILEKGMRGLSAPRIEGKFSLRASATGMIIMDSVEVPEENVLPNVSSLAGPFGCLNTARYGITWGVLGAAEFCLHTARQYALDRIQFGVPLARNQLVQKKLADMLTEITLGLHACLQLGRLKDQDKATPEMVSMLKRNNCGKALDIARQARDILGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTVGK", "length": 438, "header": "sp|Q60759-1|GCDH_MOUSE Isoform 1 of Glutaryl-CoA dehydrogenase, mitochondrial OS=Mus musculus OX=10090 GN=Gcdh PE=1 SV=2 CANONICAL=Q60759-1" }, "locus": { "chromosome": "8", "start_pos": 85620283, "end_pos": 85613612, "strand": "+", "evidence": [ { "database": "Ensembl Transcript", "id": "ENSMUST00000109745", "url": "https://www.ensembl.org/id/ENSMUST00000109745" }, { "database": "Ensembl Peptide", "id": "ENSMUSP00000105367", "url": "https://www.ensembl.org/id/ENSMUSP00000105367" } ] } } ], "glycosylation": [], "phosphorylation": [], "glycation": [], "keywords": [ "enzyme" ], "publication": [ { "title": "Comprehensive identification of phosphorylation sites in postsynaptic density preparations.", "journal": "Mol. Cell. Proteomics", "date": "2006", "authors": "Trinidad JC, Specht CG, Thalhammer A, Schoepfer R, Burlingame AL", "evidence": [ { "database": "UniProtKB", "id": "A0A0A0MQ68", "url": "https://www.uniprot.org/uniprot/A0A0A0MQ68" }, { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "reference": [ { "type": "PubMed", "id": "16452087", "url": "https://glygen.org/publication/PubMed/16452087" } ] }, { "title": "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "date": "2013", "authors": "Rardin MJ, Newman JC, Held JM, Cusack MP, Sorensen DJ, Li B, Schilling B, Mooney SD, Kahn CR, Verdin E, Gibson BW", "evidence": [ { "database": "UniProtKB", "id": "D3Z4I2", "url": "https://www.uniprot.org/uniprot/D3Z4I2" }, { "database": "UniProtKB", "id": "A0A0A0MQ68", "url": "https://www.uniprot.org/uniprot/A0A0A0MQ68" }, { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "reference": [ { "type": "PubMed", "id": "23576753", "url": "https://glygen.org/publication/PubMed/23576753" } ] }, { "title": "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways.", "journal": "Mol. Cell", "date": "2013", "authors": "Park J, Chen Y, Tishkoff DX, Peng C, Tan M, Dai L, Xie Z, Zhang Y, Zwaans BM, Skinner ME, Lombard DB, Zhao Y", "evidence": [ { "database": "UniProtKB", "id": "D3Z4I2", "url": "https://www.uniprot.org/uniprot/D3Z4I2" }, { "database": "UniProtKB", "id": "A0A0A0MQ68", "url": "https://www.uniprot.org/uniprot/A0A0A0MQ68" }, { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "reference": [ { "type": "PubMed", "id": "23806337", "url": "https://glygen.org/publication/PubMed/23806337" } ] }, { "title": "Lysine glutarylation is a protein posttranslational modification regulated by SIRT5.", "journal": "Cell Metab.", "date": "2014", "authors": "Tan M, Peng C, Anderson KA, Chhoy P, Xie Z, Dai L, Park J, Chen Y, Huang H, Zhang Y, Ro J, Wagner GR, Green MF, Madsen AS, Schmiesing J, Peterson BS, Xu G, Ilkayeva OR, Muehlbauer MJ, Braulke T, Muhlhausen C, Backos DS, Olsen CA, McGuire PJ, Pletcher SD, Lombard DB, Hirschey MD, Zhao Y", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "reference": [ { "type": "PubMed", "id": "24703693", "url": "https://glygen.org/publication/PubMed/24703693" } ] }, { "title": "Widespread and dynamic translational control of red blood cell development.", "journal": "Blood", "date": "2017", "authors": "Alvarez-Dominguez JR, Zhang X, Hu W", "evidence": [ { "database": "UniProtKB", "id": "A0A0A0MQ68", "url": "https://www.uniprot.org/uniprot/A0A0A0MQ68" } ], "reference": [ { "type": "PubMed", "id": "27899360", "url": "https://glygen.org/publication/PubMed/27899360" } ] }, { "title": "Deletion of 2-aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1.", "journal": "Journal of inherited metabolic disease", "date": "2020", "authors": "Leandro J, Dodatko T, DeVita RJ, Chen H, Stauffer B, Yu C, Houten SM", "evidence": [ { "database": "RefSeq", "id": "NP_032123", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123" } ], "reference": [ { "type": "PubMed", "id": "32567100", "url": "https://glygen.org/publication/PubMed/32567100" } ] }, { "title": "Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum-Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate.", "journal": "Molecular neurobiology", "date": "2022", "authors": "Seminotti B, Brondani M, Ribeiro RT, Leipnitz G, Wajner M", "evidence": [ { "database": "RefSeq", "id": "NP_032123", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_032123" } ], "reference": [ { "type": "PubMed", "id": "35639256", "url": "https://glygen.org/publication/PubMed/35639256" } ] }, { "title": "Cloning, structure, and chromosome localization of the mouse glutaryl-CoA dehydrogenase gene.", "journal": "Genomics", "date": "1995", "authors": "Koeller DM, DiGiulio KA, Angeloni SV, Dowler LL, Frerman FE, White RA, Goodman SI", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "reference": [ { "type": "PubMed", "id": "7490088", "url": "https://glygen.org/publication/PubMed/7490088" } ] } ], "pathway": [ { "id": "mmu:270076", "name": "", "url": "https://www.genome.jp/dbget-bin/get_linkdb?-t+pathway+mmu:270076", "resource": "KEGG Pathway" }, { "id": "R-MMU-71064", "name": "Lysine catabolism", "url": "https://www.reactome.org/PathwayBrowser/#R-MMU-71064", "resource": "Reactome", "description": "Lysine catabolism" } ], "orthologs": [ { "uniprot_canonical_ac": "Q92947-1", "protein_name": "Glutaryl-CoA dehydrogenase, mitochondrial", "gene_name": "GCDH", "tax_id": 9606, "organism": "Homo sapiens", "sequence": { "sequence": "MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK", "length": 438 }, "evidence": [ { "id": "Q60759", "url": "https://omabrowser.org/oma/group/Q60759/", "database": "OMA" } ], "common_name": "Human" } ], "go_annotation": { "categories": [ { "name": "Molecular Function", "total": 3, "go_terms": [ { "name": "glutaryl-CoA dehydrogenase activity", "id": "GO:0004361", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0004361", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "flavin adenine dinucleotide binding", "id": "GO:0050660", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0050660", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "fatty-acyl-CoA binding", "id": "GO:0000062", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0000062", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" } ] }, { "name": "Cellular Component", "total": 3, "go_terms": [ { "name": "mitochondrion", "id": "GO:0005739", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005739", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "mitochondrial inner membrane", "id": "GO:0005743", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005743", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "mitochondrial matrix", "id": "GO:0005759", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005759", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" } ] }, { "name": "Biological Process", "total": 5, "go_terms": [ { "name": "acyl-CoA metabolic process", "id": "GO:0006637", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0006637", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "fatty acid beta-oxidation using acyl-CoA dehydrogenase", "id": "GO:0033539", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0033539", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "fatty-acyl-CoA biosynthetic process", "id": "GO:0046949", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0046949", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "fatty acid oxidation", "id": "GO:0019395", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0019395", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" }, { "name": "tryptophan metabolic process", "id": "GO:0006568", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0006568", "evidence": [ { "database": "UniProtKB", "id": "Q60759", "url": "https://www.uniprot.org/uniprot/Q60759" } ], "pmid": "" } ] } ] }, "pro_annotation": [], "site_annotation": [ { "start_pos": 201, "end_pos": 203, "annotation": "n_glycosylation_sequon" }, { "start_pos": 279, "end_pos": 281, "annotation": "n_glycosylation_sequon" } ], "ptm_annotation": [], "interactions": [], "reactions": [ { "id": "13389", "name": "", "equation": "glutaryl-CoA + 2 H(+) + oxidized [electron-transfer flavoprotein] = (2E)-butenoyl-CoA + CO2 + reduced [electron-transfer flavoprotein]", "summary": "", "cellular_location": "", "input_participant_list": [], "output_participant_list": [], "enzyme_list": [ "Q92947-1", "Q60759-1", "Q54R47-1" ], "evidence": [ { "id": "13389", "database": "Rhea", "url": "https://www.rhea-db.org/rhea/13389" } ] }, { "id": "R-MMU-71046", "name": "glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2", "equation": "", "summary": "This event has been computationally inferred from an event that has been demonstrated in another species.

The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation.

More details and caveats of the event inference in Reactome. For details on PANTHER see also: http://www.pantherdb.org/about.jsp", "cellular_location": "mitochondrial matrix", "input_participant_list": [], "output_participant_list": [ "R-ALL-31649", "R-ALL-71045" ], "enzyme_list": [ "Q60759-1" ], "evidence": [ { "id": "R-MMU-71046", "database": "Reactome", "url": "https://www.reactome.org/PathwayBrowser/#R-MMU-71046" } ] } ], "reaction_participants": [ { "participant_id": "R-ALL-31649", "participant_name": "FADH2 [mitochondrial matrix]", "crossref": [ { "id": "C01352", "database": "COMPOUND", "url": "" }, { "id": "58307", "database": "ChEBI", "url": "" }, { "id": "000095099885", "database": "ZINC", "url": "" }, { "id": "C01352", "database": "COMPOUND", "url": "" }, { "id": "58307", "database": "ChEBI", "url": "" }, { "id": "000095099885", "database": "ZINC", "url": "" }, { "id": "C01352", "database": "COMPOUND", "url": "" }, { "id": "58307", "database": "ChEBI", "url": "" }, { "id": "000095099885", "database": "ZINC", "url": "" }, { "id": "C01352", "database": "COMPOUND", "url": "" }, { "id": "58307", "database": "ChEBI", "url": "" }, { "id": "000095099885", "database": "ZINC", "url": "" }, { "id": "C01352", "database": "COMPOUND", "url": "" }, { "id": "58307", "database": "ChEBI", "url": "" }, { "id": "000095099885", "database": "ZINC", "url": "" }, { "id": "C01352", "database": "COMPOUND", "url": "" }, { "id": "58307", "database": "ChEBI", "url": "" }, { "id": "000095099885", "database": "ZINC", "url": "" } ] }, { "participant_id": "R-ALL-71045", "participant_name": "crotonyl-CoA [mitochondrial matrix]", "crossref": [ { "id": "C00877", "database": "COMPOUND", "url": "" }, { "id": "57332", "database": "ChEBI", "url": "" }, { "id": "000008551257", "database": "ZINC", "url": "" }, { "id": "C00877", "database": "COMPOUND", "url": "" }, { "id": "57332", "database": "ChEBI", "url": "" }, { "id": "000008551257", "database": "ZINC", "url": "" }, { "id": "C00877", "database": "COMPOUND", "url": "" }, { "id": "57332", "database": "ChEBI", "url": "" }, { "id": "000008551257", "database": "ZINC", "url": "" }, { "id": "C00877", "database": "COMPOUND", "url": "" }, { "id": "000008551257", "database": "ZINC", "url": "" }, { "id": "57332", "database": "ChEBI", "url": "" }, { "id": "C00877", "database": "COMPOUND", "url": "" }, { "id": "57332", "database": "ChEBI", "url": "" }, { "id": "000008551257", "database": "ZINC", "url": "" } ] } ], "reaction_enzymes": [ { "uniprot_ac": "Q54R47", "gene_name": "gcdh", "activity": [ { "ec_number": "1.3.8.6", "activity": "glutaryl-CoA + 2 H(+) + oxidized [electron-transfer flavoprotein] = (2E)-butenoyl-CoA + CO2 + reduced [electron-transfer flavoprotein].", "evidence": [ { "id": "Q54R47", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q54R47" } ] }, { "ec_number": "1.3.8.6", "activity": "", "evidence": [ { "id": "Q54R47", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q54R47" } ] } ] }, { "uniprot_ac": "Q60759", "gene_name": "Gcdh", "activity": [ { "ec_number": "1.3.8.6", "activity": "glutaryl-CoA + 2 H(+) + oxidized [electron-transfer flavoprotein] = (2E)-butenoyl-CoA + CO2 + reduced [electron-transfer flavoprotein].", "evidence": [ { "id": "Q60759", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q60759" } ] }, { "ec_number": "1.3.8.6", "activity": "", "evidence": [ { "id": "Q60759", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q60759" } ] } ] }, { "uniprot_ac": "Q92947", "gene_name": "GCDH", "activity": [ { "ec_number": "1.3.8.6", "activity": "glutaryl-CoA + 2 H(+) + oxidized [electron-transfer flavoprotein] = (2E)-butenoyl-CoA + CO2 + reduced [electron-transfer flavoprotein].", "evidence": [ { "id": "Q92947", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q92947" } ] }, { "ec_number": "1.3.8.6", "activity": "", "evidence": [ { "id": "Q92947", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q92947" } ] } ] } ], "biomarkers": [], 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{ "field": "n_linked_glycans", "count": 0 }, { "field": "n_linked_glycan_sites", "count": 0 }, { "field": "o_linked_glycans", "count": 0 }, { "field": "o_linked_glycan_sites", "count": 0 } ], "sort_fields": [] }, { "table_id": "glycosylation_predicted", "table_stats": [ { "field": "total", "count": 0 }, { "field": "total_sites", "count": 0 }, { "field": "n_linked_glycans", "count": 0 }, { "field": "n_linked_glycan_sites", "count": 0 }, { "field": "o_linked_glycans", "count": 0 }, { "field": "o_linked_glycan_sites", "count": 0 } ], "sort_fields": [] }, { "table_id": "glycosylation_automatic_literature_mining", "table_stats": [ { "field": "total", "count": 0 }, { "field": "total_sites", "count": 0 }, { "field": "n_linked_glycans", "count": 0 }, { "field": "n_linked_glycan_sites", "count": 0 }, { "field": "o_linked_glycans", "count": 0 }, { "field": "o_linked_glycan_sites", "count": 0 } ], "sort_fields": [] }, { "table_id": "phosphorylation", "table_stats": [ { "field": "total", "count": 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