##gff-version 3 Q16836 UniProtKB Transit peptide 1 12 . . . Note=Mitochondrion;Ontology_term=ECO:0000305;evidence=ECO:0000305|PubMed:8687463;Dbxref=PMID:8687463 Q16836 UniProtKB Chain 13 314 . . . ID=PRO_0000007406;Note=Hydroxyacyl-coenzyme A dehydrogenase%2C mitochondrial Q16836 UniProtKB Binding site 34 39 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Binding site 57 57 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Binding site 73 73 . . . Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:10840044;Dbxref=PMID:10840044 Q16836 UniProtKB Binding site 80 80 . . . Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:10840044;Dbxref=PMID:10840044 Q16836 UniProtKB Binding site 122 122 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Binding site 127 127 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Binding site 149 149 . . . Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:10840044;Dbxref=PMID:10840044 Q16836 UniProtKB Binding site 149 149 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Binding site 173 173 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Binding site 305 305 . . . Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10231530,ECO:0000269|PubMed:10840044;Dbxref=PMID:10231530,PMID:10840044 Q16836 UniProtKB Site 170 170 . . . Note=Important for catalytic activity;Ontology_term=ECO:0000305;evidence=ECO:0000305 Q16836 UniProtKB Modified residue 80 80 . . . Note=N6-succinyllysine;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 81 81 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 81 81 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 87 87 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 87 87 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 125 125 . . . Note=N6-acetyllysine;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 127 127 . . . Note=N6-(2-hydroxyisobutyryl)lysine;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:29192674;Dbxref=PMID:29192674 Q16836 UniProtKB Modified residue 136 136 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 136 136 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 179 179 . . . Note=N6-acetyllysine;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 185 185 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0007744;evidence=ECO:0007744|PubMed:19608861;Dbxref=PMID:19608861 Q16836 UniProtKB Modified residue 185 185 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 192 192 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 192 192 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 202 202 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0007744;evidence=ECO:0007744|PubMed:19608861;Dbxref=PMID:19608861 Q16836 UniProtKB Modified residue 202 202 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 206 206 . . . Note=N6-succinyllysine;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 212 212 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 212 212 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 241 241 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0007744;evidence=ECO:0007744|PubMed:19608861;Dbxref=PMID:19608861 Q16836 UniProtKB Modified residue 241 241 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Modified residue 312 312 . . . Note=N6-acetyllysine%3B alternate;Ontology_term=ECO:0007744;evidence=ECO:0007744|PubMed:19608861;Dbxref=PMID:19608861 Q16836 UniProtKB Modified residue 312 312 . . . Note=N6-succinyllysine%3B alternate;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q61425 Q16836 UniProtKB Alternative sequence 1 1 . . . ID=VSP_016551;Note=In isoform 2. M->MGRAGLEAPPPPCGVTGTPGARGLQGRVGPRPQSLAFRGCLPRASSLPGSPRCRRRCHTM;Ontology_term=ECO:0000303;evidence=ECO:0000303|Ref.2 Q16836 UniProtKB Alternative sequence 236 236 . . . ID=VSP_016552;Note=In isoform 2 and isoform 3. R->RDFQTCGDSNSGLGFSLK;Ontology_term=ECO:0000303;evidence=ECO:0000303|Ref.2 Q16836 UniProtKB Natural variant 40 40 . . . ID=VAR_024079;Note=In HADH deficiency. A->T;Ontology_term=ECO:0000269;evidence=ECO:0000269|Ref.14;Dbxref=dbSNP:rs137853101 Q16836 UniProtKB Natural variant 57 57 . . . ID=VAR_024080;Note=In HADH deficiency. D->E;Ontology_term=ECO:0000269;evidence=ECO:0000269|Ref.14;Dbxref=dbSNP:rs137853102 Q16836 UniProtKB Natural variant 57 57 . . . ID=VAR_083648;Note=Found in a patient with Reye-like syndrome. Does not affect 3-hydroxyacyl-CoA dehydrogenase activity. Increases KM value for NADH. Does not affect dimerization. D->G;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:16725361;Dbxref=PMID:16725361 Q16836 UniProtKB Natural variant 86 86 . . . ID=VAR_026764;Note=L->P;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:15489334,ECO:0000269|PubMed:8687463,ECO:0000269|Ref.2,ECO:0000269|Ref.3;Dbxref=dbSNP:rs4956145,PMID:15489334,PMID:8687463 Q16836 UniProtKB Natural variant 152 152 . . . ID=VAR_055701;Note=Q->H;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8687463;Dbxref=dbSNP:rs1051519,PMID:8687463 Q16836 UniProtKB Natural variant 226 226 . . . ID=VAR_083649;Note=Found in a patient with Reye-like syndrome%3B loss of 3-hydroxyacyl-CoA dehydrogenase activity. Does not affect dimerization. Y->H;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:16725361;Dbxref=dbSNP:rs146036912,PMID:16725361 Q16836 UniProtKB Natural variant 258 258 . . . ID=VAR_024081;Note=In HHF4%3B loss of 3-hydroxyacyl-CoA dehydrogenase activity. P->L;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:11489939;Dbxref=dbSNP:rs137853103,PMID:11489939 Q16836 UniProtKB Helix 1 11 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:3RQS Q16836 UniProtKB Helix 14 22 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:3RQS Q16836 UniProtKB Beta strand 29 33 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 37 48 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 52 56 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 60 79 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 82 84 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 86 98 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 100 104 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 106 109 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 110 112 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:3HAD Q16836 UniProtKB Beta strand 114 118 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 124 134 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Turn 135 137 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 143 146 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 149 151 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 153 157 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 163 165 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 166 171 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Turn 175 177 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 180 184 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 191 203 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 207 211 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Turn 215 218 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 219 235 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 241 252 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 258 265 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 267 279 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Turn 280 283 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 285 287 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Helix 291 298 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Turn 304 307 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Beta strand 308 312 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:1F0Y Q16836 UniProtKB Sequence conflict 41 41 . . . Note=L->P;Ontology_term=ECO:0000305;evidence=ECO:0000305 Q16836 UniProtKB Sequence conflict 56 56 . . . Note=R->H;Ontology_term=ECO:0000305;evidence=ECO:0000305