Hydroxymethylglutaryl-CoA lyase, mitochondrial (HL) (HMG-CoA lyase) (EC 4.1.3.4) (3-hydroxy-3-methylglutarate-CoA lyase)
1_MAAMR 6_ KALPR 11_ RLVGL 16_ ASLRA 21_ VSTSS 26_ MGTLP 31_ KRVKI 36_ VEVGP 41_ RDGLQ 46_ NEKNI 51_ VSTPV 56_ KIKLI 61_ DMLSE 66_ AGLSV 71_ IETTS 76_ FVSPK 81_ WVPQM 86_ GDHTE 91_ VLKGI 96_ QKFPG 101_ INYPV 106_ LTPNL 111_ KGFEA 116_ AVAAG 121_ AKEVV 126_ IFGAA 131_ SELFT 136_ KKNIN 141_ CSIEE 146_ SFQRF 151_ DAILK 156_ AAQSA 161_ NISVR 166_ GYVSC 171_ ALGCP 176_ YEGKI 181_ SPAKV 186_ AEVTK 191_ KFYSM 196_ GCYEI 201_ SLGDT 206_ IGVGT 211_ PGIMK 216_ DMLSA 221_ VMQEV 226_ PLAAL 231_ AVHCH 236_ DTYGQ 241_ ALANT 246_ LMALQ 251_ MGVSV 256_ VDSSV 261_ AGLGG 266_ CPYAQ 271_ GASGN 276_ LATED 281_ LVYML 286_ EGLGI 291_ HTGVN 296_ LQKLL 301_ EAGNF 306_ ICQAL 311_ NRKTS 316_SKVAQ
1: Mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism