Succinate:Coenzyme-A Transferase (pig heart)

Succinate:Coenzyme-A Transferase Deficiency: A Structural View of Pathogenic Mutations

SUCCINYL-COA\:3-KETOACID-COENZYME A TRANSFERASE

SUCCINYL COA\:3-OXOACID COA-TRANSFERASE, OXCT, SCOT

TKFYTDAVEAVKDIPNGATVLVGGFGLCGIPENLIGALLKTGVKELTAVSNNAGVDNFGLGLLLQSKQIKRMISSYVGEN
AEFERQYLAGELEVELTPQGTLAERIRAGGAGVPAFYTSTGYGTLVQEGGSPIKYNKDGSIAIASKPREVREFNGQHFIL
EEAIRGDFALVKAWKADQAGNVTFRKSARNFNLPMCKAAETTVVEVEEIVDIGSFAPEDIHIPKIYVHRLVKGEKYEKRI
ERLSVRKEEDVKTRSGKLGDNVRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNMTVHLQSENGILGLGPYPLQNEV
DADLINAGKETVTVLPGASYFSSDESFAMIRGGHVNLTMLGAMQVSKYGDLANWMIPGKLVKGMGGAMDLVSSAKTKVVV
TMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDRKKGLTLIELWEGLTVDDIKKSTGCDFAVSPKLIPMQQVT
T

SHEET
THE SHEET STRUCTURE OF THIS MOLECULE IS BIFURCATED. IN
ORDER TO REPRESENT THIS FEATURE IN THE SHEET RECORDS BELOW,
TWO SHEETS ARE DEFINED.

KEY ENZYME FOR KETONE BODY CATABOLISM. TRANSFERS THE COA MOIETY FROM SUCCINATE TO ACETOACETATE. FORMATION OF THE ENZYME-COA INTERMEDIATE PROCEEDS VIA AN UNSTABLE ANHYDRIDE SPECIES FORMED BETWEEN THE CARBOXYLATE GROUPS OF THE ENZYME AND SUBSTRATE.

RESIDUES IN THE SERVERAL REGIONS WERE NOT VISIBLE IN ELECTRON DENSITY MAPS AND WERE NOT MODELLED: A/B287-299,C286-299, D283-299, B/C401-419, B/C441-465

CONEZYME-A, TRANSFERASE, MAD, PATHOGENIC MUTATIONS