{ "uniprot_canonical_ac": "P29266-1", "uniprot_ac": "P29266", "uniprot_id": "3HIDH_RAT", "mass": { "chemical_mass": 35303.0, "monoisotopic_mass": 35303.0 }, "refseq": { "ac": "NP_071579", "name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial precursor", "summary": "", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579" }, "gene_names": [ { "name": "Hibadh", "resource": "UniProtKB", "type": "recommended", "url": "https://www.uniprot.org/uniprot/P29266", "id": "P29266" }, { "name": "Hibadh", "resource": "RefSeq", "type": "synonym", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579", "id": "NP_071579" } ], "protein_names": [ { "name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial", "resource": "UniProtKB", "type": "recommended", "url": "", "id": "P29266" }, { "name": "1.1.1.31", "resource": "UniProtKB", "type": "synonym", "url": "", "id": "P29266" }, { "name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial precursor", "resource": "RefSeq", "type": "synonym", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579", "id": "NP_071579" }, { "name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial", "resource": "RefSeq", "type": "synonym", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579", "id": "NP_071579" }, { "name": "HIBADH", "resource": "UniProtKB", "type": "synonym", "url": "", "id": "P29266" } ], "function": [ { "annotation": "The expression of 3-hydroxyisobutyrate dehydrogenase in neural cells demonstrates their potential to utilize valine imported into the brain for the generation of energy.", "evidence": [ { "database": "RefSeq", "id": "NP_071579", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579" }, { "database": "PubMed", "id": "18284611", "url": "https://glygen.org/publication/PubMed/18284611" } ] } ], "disease": [], "snv": [], "mutagenesis": [ { "start_pos": 208, "end_pos": 208, "sequence_org": "K", "sequence_mut": "A", "comment": "Complete loss of activity.", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" }, { "database": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] }, { "start_pos": 208, "end_pos": 208, "sequence_org": "K", "sequence_mut": "H", "comment": "Complete loss of activity.", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" }, { "database": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] }, { "start_pos": 208, "end_pos": 208, "sequence_org": "K", "sequence_mut": "N", "comment": "Complete loss of activity.", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" }, { "database": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] }, { "start_pos": 208, "end_pos": 208, "sequence_org": "K", "sequence_mut": "R", "comment": "Complete loss of activity.", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" }, { "database": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] }, { "start_pos": 212, "end_pos": 212, "sequence_org": "N", "sequence_mut": "Q", "comment": "Decrease in activity.", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" }, { "database": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] }, { "start_pos": 68, "end_pos": 68, "sequence_org": "D", "sequence_mut": "R", "comment": "Decrease of activity with NAD, increase of activity with NADP.", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" }, { "database": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] } ], "expression_tissue": [], "expression_disease": [], "species": [ { "name": "Rattus norvegicus", "taxid": 10116, "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "common_name": "Rat" } ], "gene": [ { "name": "Hibadh", "url": "https://www.uniprot.org/uniprot/P29266", "locus": { "chromosome": "4", "start_pos": 81649175, "end_pos": 81780617, "strand": "+", "evidence": [ { "database": "Ensembl Gene", "id": "ENSRNOG00000008063", "url": "https://www.ensembl.org/id/ENSRNOG00000008063" } ] } } ], "crossref": [ { "id": "ENSRNOG00000008063", "url": "https://bgee.org/?page=gene&gene_id=ENSRNOG00000008063", "database": "Bgee", "categories": [ "Gene Expression" ] }, { "id": "63938", "url": "https://www.ncbi.nlm.nih.gov/gene/63938", "database": "GeneID", "categories": [ "Genome Annotation" ] }, { "id": "P29266", "url": "https://www.ebi.ac.uk/intact/interactors/id:P29266*", "database": "IntAct", "categories": [ "Other" ] }, { "id": "IPR002204", "url": "https://www.ebi.ac.uk/interpro/entry/IPR002204", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR006115", "url": "https://www.ebi.ac.uk/interpro/entry/IPR006115", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR008927", "url": "https://www.ebi.ac.uk/interpro/entry/IPR008927", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR011548", "url": "https://www.ebi.ac.uk/interpro/entry/IPR011548", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR013328", "url": "https://www.ebi.ac.uk/interpro/entry/IPR013328", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR015815", "url": "https://www.ebi.ac.uk/interpro/entry/IPR015815", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR029154", "url": "https://www.ebi.ac.uk/interpro/entry/IPR029154", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "IPR036291", "url": "https://www.ebi.ac.uk/interpro/entry/IPR036291", "database": "InterPro", "categories": [ "Protein Classification" ] }, { "id": "rno:63938", "url": "https://www.genome.jp/dbget-bin/get_linkdb?-t+pathway+rno:63938", "database": "KEGG Pathway", "categories": [ "Enzyme and Pathway" ] }, { "id": "P29266", "url": "https://oglcnac.org/atlas/detail/P29266", "database": "O-GlcNAc Atlas", "categories": [ "Glycoproteomics" ] }, { "id": "P29266", "url": "https://www.oglcnac.mcw.edu/search/?query_protein=P29266", "database": "O-GlcNAc Database", "categories": [ "Glycoproteomics" ] }, { "id": "MGKKVWH", "url": "https://omabrowser.org/oma/group/MGKKVWH/", "database": "OMA", "categories": [ "Protein Classification" ] }, { "id": "203032at2759", "url": "http://www.orthodb.org/?query=203032at2759", "database": "OrthoDB", "categories": [ "Protein Classification" ] }, { "id": "PTHR22981", "url": "http://www.pantherdb.org/panther/family.do?clsAccession=PTHR22981", "database": "PANTHER", "categories": [ "Protein Classification" ] }, { "id": "PTHR22981:SF7", "url": "http://www.pantherdb.org/panther/family.do?clsAccession=PTHR22981:SF7", "database": "PANTHER", "categories": [ "Protein Classification" ] }, { "id": "PF03446", "url": "http://pfam.xfam.org/family/PF03446", "database": "Pfam", "categories": [ "Protein Classification" ] }, { "id": "PF14833", "url": "http://pfam.xfam.org/family/PF14833", "database": "Pfam", "categories": [ "Protein Classification" ] }, { "id": "PR_P29266", "url": "http://purl.obolibrary.org/obo/PR_P29266", "database": "PRO", "categories": [ "Protein" ] }, { "id": "R-RNO-70895", "url": "https://www.reactome.org/PathwayBrowser/#R-RNO-70895", "database": "Reactome", "categories": [ "Protein Classification" ] }, { "id": "NP_071579", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579", "database": "RefSeq", "categories": [ "Protein" ] }, { "id": "708399", "url": "http://rgd.mcw.edu/tools/genes/genes_view.cgi?id=708399", "database": "RGD", "categories": [ "Organism-specific" ] }, { "id": "17681", "url": "https://www.rhea-db.org/rhea/17681", "database": "Rhea", "categories": [ "Enzyme and Pathway" ] }, { "id": "A0A8I5ZVQ3", "url": "https://www.uniprot.org/uniprot/A0A8I5ZVQ3", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "A0A8I5ZVQ3-1", "url": "https://www.uniprot.org/uniprot/A0A8I5ZVQ3-1", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "A0A8I6AQ40", "url": "https://www.uniprot.org/uniprot/A0A8I6AQ40", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "A0A8I6AQ40-1", "url": "https://www.uniprot.org/uniprot/A0A8I6AQ40-1", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "A0A8L2Q4S5", "url": "https://www.uniprot.org/uniprot/A0A8L2Q4S5", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "A0A8L2Q4S5-1", "url": "https://www.uniprot.org/uniprot/A0A8L2Q4S5-1", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266", "database": "UniProtKB", "categories": [ "Protein" ] }, { "id": "P29266-1", "url": "https://www.uniprot.org/uniprot/P29266-1", "database": "UniProtKB", "categories": [ "Protein" ] } ], "sequence": { "sequence": "MAASLGFRGAASGLRYWSGRRRPVGSLAAVCSRSMASKTPVGFIGLGNMGNPMAKNLIKHGYPLILYDVFPDVCKEFKEAGEQVASSPADVAEKADRIITMLPSSMNSIEVYSGANGILKKVKKGSLLIDSSTIDPSVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVENEFAAAQELLGCMGSNVLYCGAVGSGQSAKICNNMLLAISMIGTAEAMNLGIRSGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMDGVPSSNNYQGGFGTTLMAKDLGLAQDSATSTKTPILLGSVAHQIYRMMCSKGYSKKDFSSVFQYLREEETF", "length": 335, "header": "sp|P29266-1|3HIDH_RAT Isoform 1 of 3-hydroxyisobutyrate dehydrogenase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Hibadh PE=1 SV=3 CANONICAL=P29266-1" }, "isoforms": [ { "isoform_ac": "A0A8I5ZVQ3-1", "url": "", "sequence": { "sequence": "MHVCVPQVYFLFINVLKSVCSRSMASKTPVGFIGLGNMGNPMAKNLIKHGYPLILYDVFPDVCKEFKEAGEQVASSPADVAEKADRIITMLPSSMNSIEVYSGANGILKKVKKGSLLIDSSTIDPSVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVENEFAAAQELLGCMGSNVLYCGAVGSGQSAKICNNMLLAISMIGTAEAMNLGIRSGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMDGVPSSNNYQGGFGTTLMAKDLGLAQDSATSTKTPILLGSVAHQIYRMMCSKGYSKKDFSSVFQYLREEETF", "length": 324, "header": "tr|A0A8I5ZVQ3-1|3HIDH_RAT Computationally mapped isoform of 3-hydroxyisobutyrate dehydrogenase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Hibadh PE=1 SV=1 CANONICAL=P29266-1" }, "locus": { "chromosome": "4", "start_pos": 81737495, "end_pos": 81649896, "strand": "+", "evidence": [ { "database": "Ensembl Transcript", "id": "ENSRNOT00000099996.1", "url": "https://www.ensembl.org/id/ENSRNOT00000099996.1" }, { "database": "Ensembl Peptide", "id": "ENSRNOP00000082691.1", "url": "https://www.ensembl.org/id/ENSRNOP00000082691.1" } ] } }, { "isoform_ac": "A0A8I6AQ40-1", "url": "", "sequence": { "sequence": "MDNTVPFISDLQYCQSMASKTPVGFIGLGNMGNPMAKNLIKHGYPLILYDVFPDVCKEFKEAGEQVASSPADVAEKADRIITMLPSSMNSIEVYSGANGILKKVKKGSLLIDSSTIDPSVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVENEFAAAQELLGCMGSNVLYCGAVGSGQSAKICNNMLLAISMIGTAEAMNLGIRSGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMDGVPSSNNYQGGFGTTLMAKDLGLAQDSATSTKTPILLGSVAHQIYRMMCSKGYSKKDFSSVFQYLREEETF", "length": 317, "header": "tr|A0A8I6AQ40-1|3HIDH_RAT Computationally mapped isoform of 3-hydroxyisobutyrate dehydrogenase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Hibadh PE=1 SV=1 CANONICAL=P29266-1" }, "locus": { "chromosome": "4", "start_pos": 81780556, "end_pos": 81649896, "strand": "+", "evidence": [ { "database": "Ensembl Transcript", "id": "ENSRNOT00000120276.1", "url": "https://www.ensembl.org/id/ENSRNOT00000120276.1" }, { "database": "Ensembl Peptide", "id": "ENSRNOP00000095911.1", "url": "https://www.ensembl.org/id/ENSRNOP00000095911.1" } ] } }, { "isoform_ac": "A0A8L2Q4S5-1", "url": "", "sequence": { "sequence": "MAASLGFRGAASGLRYWSGRRRPVGSLAAVCSRSMASKTPVGFIGLGNMGNPMAKNLIKHGYPLILYDVFPDVCKEFKEAGEQVASSPADVAEKADRIITMLPSSMNSIEVYSGANGILKKVKKGSLLIDSSTIDPSVSKELAKEVEKMGAVFMDAPVSGGAARSGNLTFMVGGVENEFAAAQELLGCMGSNVLYCGAVGSGQSAKICNNMLLAISMIGTAEAMNLGIRSGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMDGVPSSNNYQGGFGTTLMAKDLGLAQDSATSTKTPILLGSVAHQIYRMMCSKGYSKKDFSSVFQYLREEETF", "length": 333, "header": "tr|A0A8L2Q4S5-1|3HIDH_RAT Computationally mapped isoform of 3-hydroxyisobutyrate dehydrogenase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Hibadh PE=1 SV=1 CANONICAL=P29266-1" }, "locus": { "chromosome": "4", "start_pos": 81747212, "end_pos": 81649896, "strand": "+", "evidence": [ { "database": "Ensembl Transcript", "id": "ENSRNOT00000011069.7", "url": "https://www.ensembl.org/id/ENSRNOT00000011069.7" }, { "database": "Ensembl Peptide", "id": "ENSRNOP00000011069.5", "url": "https://www.ensembl.org/id/ENSRNOP00000011069.5" } ] } }, { "isoform_ac": "P29266-1", "url": "", "sequence": { "sequence": "MAASLGFRGAASGLRYWSGRRRPVGSLAAVCSRSMASKTPVGFIGLGNMGNPMAKNLIKHGYPLILYDVFPDVCKEFKEAGEQVASSPADVAEKADRIITMLPSSMNSIEVYSGANGILKKVKKGSLLIDSSTIDPSVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVENEFAAAQELLGCMGSNVLYCGAVGSGQSAKICNNMLLAISMIGTAEAMNLGIRSGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMDGVPSSNNYQGGFGTTLMAKDLGLAQDSATSTKTPILLGSVAHQIYRMMCSKGYSKKDFSSVFQYLREEETF", "length": 335, "header": "sp|P29266-1|3HIDH_RAT Isoform 1 of 3-hydroxyisobutyrate dehydrogenase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Hibadh PE=1 SV=3 CANONICAL=P29266-1" }, "locus": {} } ], "glycosylation": [ { "glytoucan_ac": "G49108TO", "type": "O-linked", "subtype": "O-GlcNAcylation", "site_category": "reported_with_glycan", "site_seq": "S", "relation": "attached", "comment": "O-GlcNAcylation", "start_pos": 159, "start_aa": "Ser", "end_pos": 159, "end_aa": "Ser", "residue": "Ser", "site_lbl": "Ser159", "evidence": [ { "id": "P29266", "database": "O-GlcNAc Database", "url": "https://www.oglcnac.mcw.edu/search/?query_protein=P29266" }, { "id": "27213235", "database": "PubMed", "url": "https://glygen.org/publication/PubMed/27213235" }, { "id": "26446791", "database": "PubMed", "url": "https://glygen.org/publication/PubMed/26446791" }, { "id": "P29266", "database": "O-GlcNAc Atlas", "url": "https://oglcnac.org/atlas/detail/P29266" } ] }, { "glytoucan_ac": "G49108TO", "type": "O-linked", "subtype": "O-GlcNAcylation", "site_category": "reported_with_glycan", "site_seq": "S", "relation": "attached", "comment": "O-GlcNAcylation", "start_pos": 303, "start_aa": "Ser", "end_pos": 303, "end_aa": "Ser", "residue": "Ser", "site_lbl": "Ser303", "evidence": [ { "id": "P29266", "database": "O-GlcNAc Database", "url": "https://www.oglcnac.mcw.edu/search/?query_protein=P29266" }, { "id": "27213235", "database": "PubMed", "url": "https://glygen.org/publication/PubMed/27213235" }, { "id": "26446791", "database": "PubMed", "url": "https://glygen.org/publication/PubMed/26446791" }, { "id": "P29266", "database": "O-GlcNAc Atlas", "url": "https://oglcnac.org/atlas/detail/P29266" } ] } ], "phosphorylation": [], "glycation": [], "keywords": [ "enzyme", "glycoprotein", "glycoprotein_reported_with_glycan" ], "publication": [ { "title": "Expression of 3-hydroxyisobutyrate dehydrogenase in cultured neural cells.", "journal": "Journal of neurochemistry", "date": "2008", "authors": "Murin R, Schaer A, Kowtharapu BS, Verleysdonk S, Hamprecht B", "evidence": [ { "database": "RefSeq", "id": "NP_071579", "url": "https://www.ncbi.nlm.nih.gov/protein/NP_071579" } ], "reference": [ { "type": "PubMed", "id": "18284611", "url": "https://glygen.org/publication/PubMed/18284611" } ] }, { "title": "O-GlcNAcomic Profiling Identifies Widespread O-Linked beta-N-Acetylglucosamine Modification (O-GlcNAcylation) in Oxidative Phosphorylation System Regulating Cardiac Mitochondrial Function.", "journal": "The Journal of biological chemistry", "date": "2015", "authors": "Ma J, Liu T, Wei AC, Banerjee P, O'Rourke B, Hart GW", "evidence": [ { "database": "O-GlcNAc Database", "id": "P29266", "url": "https://www.oglcnac.mcw.edu/search/?query_protein=P29266" }, { "database": "O-GlcNAc Atlas", "id": "P29266", "url": "https://oglcnac.org/atlas/detail/P29266" } ], "reference": [ { "type": "PubMed", "id": "26446791", "url": "https://glygen.org/publication/PubMed/26446791" } ] }, { "title": "Cloning and sequence analysis of a cDNA for 3-hydroxyisobutyrate dehydrogenase. Evidence for its evolutionary relationship to other pyridine nucleotide-dependent dehydrogenases.", "journal": "J. Biol. Chem.", "date": "1989", "authors": "Rougraff PM, Zhang B, Kuntz MJ, Harris RA, Crabb DW", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "reference": [ { "type": "PubMed", "id": "2647728", "url": "https://glygen.org/publication/PubMed/2647728" } ] }, { "title": "Comparative Proteomics Reveals Dysregulated Mitochondrial O-GlcNAcylation in Diabetic Hearts.", "journal": "Journal of proteome research", "date": "2016", "authors": "Ma J, Banerjee P, Whelan SA, Liu T, Wei AC, Ramirez-Correa G, McComb ME, Costello CE, O'Rourke B, Murphy A, Hart GW", "evidence": [ { "database": "O-GlcNAc Database", "id": "P29266", "url": "https://www.oglcnac.mcw.edu/search/?query_protein=P29266" }, { "database": "O-GlcNAc Atlas", "id": "P29266", "url": "https://oglcnac.org/atlas/detail/P29266" } ], "reference": [ { "type": "PubMed", "id": "27213235", "url": "https://glygen.org/publication/PubMed/27213235" } ] }, { "title": "Structural and mechanistic similarities of 6-phosphogluconate and 3-hydroxyisobutyrate dehydrogenases reveal a new enzyme family, the 3-hydroxyacid dehydrogenases.", "journal": "FEBS Lett.", "date": "1996", "authors": "Hawes JW, Harper ET, Crabb DW, Harris RA", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "reference": [ { "type": "PubMed", "id": "8766712", "url": "https://glygen.org/publication/PubMed/8766712" } ] } ], "pathway": [ { "id": "rno:63938", "name": "", "url": "https://www.genome.jp/dbget-bin/get_linkdb?-t+pathway+rno:63938", "resource": "KEGG Pathway" }, { "id": "R-RNO-70895", "name": "Branched-chain amino acid catabolism", "url": "https://www.reactome.org/PathwayBrowser/#R-RNO-70895", "resource": "Reactome", "description": "Branched-chain amino acid catabolism" } ], "orthologs": [ { "uniprot_canonical_ac": "P31937-1", "protein_name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial", "gene_name": "HIBADH", "tax_id": 9606, "organism": "Homo sapiens", "sequence": { "sequence": "MAASLRLLGAASGLRYWSRRLRPAAGSFAAVCSRSVASKTPVGFIGLGNMGNPMAKNLMKHGYPLIIYDVFPDACKEFQDAGEQVVSSPADVAEKADRIITMLPTSINAIEAYSGANGILKKVKKGSLLIDSSTIDPAVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVEDEFAAAQELLGCMGSNVVYCGAVGTGQAAKICNNMLLAISMIGTAEAMNLGIRLGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMDGVPSANNYQGGFGTTLMAKDLGLAQDSATSTKSPILLGSLAHQIYRMMCAKGYSKKDFSSVFQFLREEETF", "length": 336 }, "evidence": [ { "id": "45232234", "url": "http://www.informatics.jax.org/homology/45232234", "database": "MGI" } ], "common_name": "Human" }, { "uniprot_canonical_ac": "Q99L13-1", "protein_name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial", "gene_name": "Hibadh", "tax_id": 10090, "organism": "Mus musculus", "sequence": { "sequence": "MAASLGFRGAASGLWYWSGRRRPVGSLAAVCSRSMASKTPVGFIGLGNMGNPMAKNLMKHGYPLILYDVFPDVCKEFKEAGEQVASSPAEVAEKADRIITMLPSSMNAVEVYSGANGILKKVKKGSLLIDSSTIDPSVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVEDEFAAAQELLECMGSNVVYCGAVGTGQSAKICNNMLLAISMIGTAEAMNLGIRSGLDPKLLAKILNMSSGRCWSSDTYNPVPGVMHGVPSSNNYQGGFGTTLMAKDLGLAQDSATSTKTPILLGSLAHQIYRMMCSKGYSKKDFSSVFQYLREEEPF", "length": 335 }, "evidence": [ { "id": "45232234", "url": "http://www.informatics.jax.org/homology/45232234", "database": "MGI" } ], "common_name": "Mouse" } ], "go_annotation": { "categories": [ { "name": "Molecular Function", "total": 3, "go_terms": [ { "name": "NAD binding", "id": "GO:0051287", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0051287", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "pmid": "" }, { "name": "3-hydroxyisobutyrate dehydrogenase activity", "id": "GO:0008442", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0008442", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "pmid": "" }, { "name": "NADP binding", "id": "GO:0050661", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0050661", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "pmid": "" } ] }, { "name": "Biological Process", "total": 1, "go_terms": [ { "name": "valine catabolic process", "id": "GO:0006574", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0006574", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "pmid": "" } ] }, { "name": "Cellular Component", "total": 1, "go_terms": [ { "name": "mitochondrion", "id": "GO:0005739", "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005739", "evidence": [ { "database": "UniProtKB", "id": "P29266", "url": "https://www.uniprot.org/uniprot/P29266" } ], "pmid": "" } ] } ] }, "pro_annotation": [], "site_annotation": [ { "start_pos": 169, "end_pos": 171, "annotation": "n_glycosylation_sequon" }, { "start_pos": 244, "end_pos": 246, "annotation": "n_glycosylation_sequon" } ], "ptm_annotation": [], "interactions": [], "reactions": [ { "id": "17681", "name": "", "equation": "3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + H(+) + NADH", "summary": "", "cellular_location": "", "input_participant_list": [], "output_participant_list": [], "enzyme_list": [ "P29266-1", "P31937-1", "Q99L13-1", "Q54CX6-1", "Q9V8M5-1" ], "evidence": [ { "id": "17681", "database": "Rhea", "url": "https://www.rhea-db.org/rhea/17681" } ] }, { "id": "R-RNO-508473", "name": "methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+", "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-573404" ], "enzyme_list": [ "P29266-1" ], "evidence": [ { "id": "R-RNO-508473", "database": "Reactome", "url": "https://www.reactome.org/PathwayBrowser/#R-RNO-508473" } ] }, { "id": "R-RNO-70885", "name": "beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+", "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": [], "enzyme_list": [ "P29266-1" ], "evidence": [ { "id": "R-RNO-70885", "database": "Reactome", "url": "https://www.reactome.org/PathwayBrowser/#R-RNO-70885" } ] } ], "reaction_participants": [ { "participant_id": "R-ALL-573404", "participant_name": "bHIBA [mitochondrial matrix]", "crossref": [ { "id": "11805", "database": "ChEBI", "url": "" }, { "id": "000000895528", "database": "ZINC", "url": "" }, { "id": "000000895526", "database": "ZINC", "url": "" }, { "id": "11805", "database": "ChEBI", "url": "" }, { "id": "000000895526", "database": "ZINC", "url": "" }, { "id": "000000895528", "database": "ZINC", "url": "" }, { "id": "11805", "database": "ChEBI", "url": "" }, { "id": "000000895526", "database": "ZINC", "url": "" }, { "id": "000000895528", "database": "ZINC", "url": "" }, { "id": "11805", "database": "ChEBI", "url": "" }, { "id": "000000895526", "database": "ZINC", "url": "" }, { "id": "000000895528", "database": "ZINC", "url": "" }, { "id": "11805", "database": "ChEBI", "url": "" }, { "id": "000000895526", "database": "ZINC", "url": "" }, { "id": "000000895528", "database": "ZINC", "url": "" }, { "id": "11805", "database": "ChEBI", "url": "" }, { "id": "000000895526", "database": "ZINC", "url": "" }, { "id": "000000895528", "database": "ZINC", "url": "" } ] } ], "reaction_enzymes": [ { "uniprot_ac": "P29266", "gene_name": "Hibadh", "activity": [ { "ec_number": "1.1.1.31", "activity": "3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + H(+) + NADH.", "evidence": [ { "id": "P29266", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/P29266" } ] }, { "ec_number": "1.1.1.31", "activity": "", "evidence": [ { "id": "P29266", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/P29266" } ] } ] }, { "uniprot_ac": "P31937", "gene_name": "HIBADH", "activity": [ { "ec_number": "1.1.1.31", "activity": "3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + H(+) + NADH.", "evidence": [ { "id": "P31937", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/P31937" } ] }, { "ec_number": "1.1.1.31", "activity": "", "evidence": [ { "id": "P31937", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/P31937" } ] } ] }, { "uniprot_ac": "Q54CX6", "gene_name": "hibA", "activity": [ { "ec_number": "1.1.1.31", "activity": "3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + H(+) + NADH.", "evidence": [ { "id": "Q54CX6", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q54CX6" } ] }, { "ec_number": "1.1.1.31", "activity": "", "evidence": [ { "id": "Q54CX6", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q54CX6" } ] } ] }, { "uniprot_ac": "Q99L13", "gene_name": "Hibadh", "activity": [ { "ec_number": "1.1.1.31", "activity": "3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + H(+) + NADH.", "evidence": [ { "id": "Q99L13", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q99L13" } ] }, { "ec_number": "1.1.1.31", "activity": "", "evidence": [ { "id": "Q99L13", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q99L13" } ] } ] }, { "uniprot_ac": "Q9V8M5", "gene_name": "", "activity": [ { "ec_number": "1.1.1.31", "activity": "(S)-3-hydroxy-2-methylpropanoate + NAD(+) = (S)-2-methyl-3-oxopropanoate + H(+) + NADH.", "evidence": [ { "id": "Q9V8M5", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q9V8M5" } ] }, { "ec_number": "1.1.1.31", "activity": "", "evidence": [ { "id": "Q9V8M5", "database": "UniProtKB", "url": "https://www.uniprot.org/uniprot/Q9V8M5" } ] } ] } ], "biomarkers": [], "synthesized_glycans": [], "cluster_types": [ { "name": "isoformset.uniprotkb", "label": "Isoforms" }, { "name": "homologset.mgi", "label": "MGI Homologs" } ], "section_stats": [ { "table_id": "glycosylation", "table_stats": [ { "field": "total", "count": 2 }, { "field": "total_sites", "count": 2 }, { "field": "n_linked_glycans", "count": 0 }, { "field": "n_linked_glycan_sites", "count": 0 }, { "field": "o_linked_glycans", "count": 1 }, { "field": "o_linked_glycan_sites", "count": 2 } ], "sort_fields": [ "glytoucan_ac", "type", "subtype", "site_category", "site_seq", "relation", "comment", "start_pos", "start_aa", "end_pos", "end_aa", "residue", "site_lbl" ] }, { "table_id": "glycosylation_reported", "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_reported_with_glycan", "table_stats": [ { "field": "total", "count": 2 }, { "field": "total_sites", "count": 2 }, { "field": "n_linked_glycans", "count": 0 }, { "field": "n_linked_glycan_sites", "count": 0 }, { "field": "o_linked_glycans", "count": 1 }, { "field": "o_linked_glycan_sites", "count": 2 } ], "sort_fields": [ "glytoucan_ac", "type", "subtype", "site_category", "site_seq", "relation", "comment", "start_pos", "start_aa", "end_pos", "end_aa", "residue", "site_lbl" ] }, { "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": 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