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Febrina Meutiawati 10507039

Presentasi Enzimologi Acyl-CoA Dehydrogenase (ACD). Febrina Meutiawati 10507039. Oksidasi - β asam lemak. Acyl-CoA Dehydrogenase. Merupakan Flavoenzim yang mengkatalisis reaksi α , β - dehidrogenasi turunan asil-KoA.

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Febrina Meutiawati 10507039

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  1. PresentasiEnzimologi • Acyl-CoADehydrogenase • (ACD) FebrinaMeutiawati 10507039

  2. Oksidasi-βasamlemak

  3. Acyl-CoADehydrogenase MerupakanFlavoenzim yang mengkatalisisreaksiα,β-dehidrogenasiturunanasil-KoA Terdapat 9 member dalamkeluargaasil-KoAdehidrogenase, berperandalamoksidasiasamlemakdanasam amino Oksidasiasamlemak : SCAD, MCAD, LCAD, VLCAD Oksidasiasam amino : IVD, S/BCAD, GCD • MCAD (Medium Chain Acyl-CoADehydrogenase)

  4. Termasukkedalamkelasenzimoksidoreduktase Tata Namamenurut NC-IUBMB • SCAD (EC.1.3.99.2) • MCAD(EC.1.3.99.3) • IVD (EC.1.3.99.10) • LCAD (EC.1.3.99.13)

  5. Acyl-CoAdehydrogenasediisolasidaribouvine livermitokondriamenggunakanmetodeacetone dry powder. Pemurniandilakukandengan PresipitasiAmoniumSulfat Gel Filtrasi KolomkromatografiPenukar Ion DEAE-selulosa Elektroforesispreparatif ISOLASI danpemurnian

  6. Struktur 3D MCAD • Homotetramer • dimerdaridimer • d = 90Å • Memilikisisipengikatanuntuk FAD danasil-KoA

  7. Struktur Pita GCD (Glutaryl-CoADehydrogenase)

  8. Perbedaan ACD OksidasiAsamLemakdengan ACD OksidasiAsam Amino

  9. MekanismeReaksi • pH 7,6 1˚C

  10. Transfer Elektron

  11. Kinetika

  12. MCAD hexadecanoyl-CoA all-cis-4,7,10,13-hexdecanoyl-Co LCAD

  13. InhibisiIn vitro Subsratpalmitoyl-coa

  14. Inhibisi SubsratButyryl-coa

  15. Inhibisi Subsratisovaleryl-coa

  16. Dicarboxylicaciduriamerupakanpenyakit yang disebabkanadanyagangguanmetabolisme, yaituβ-oksidasiasamlemak C6-C10. Defisiensi ACD • Ditunjukkandenganadanyaepisodic hypoglycemia yang tidakdiikutidengan ketosis. • Adapeningkatanjumlah C6-C10ω-asamdikarboksilatdalamurin. • Adanyamutasipadarantai GCD dimana LYS304 digantiolehresiduglutamatmenghasilkankekacauanmetabolisme. • Adanyamutasipada terminal karboksilpada GCD menyebabkanglutaricacidemiatipe 1.

  17. Fu Zhuji, Ming Wang, Rosemary Paschke, K. SudhindraRao, Frank E. Frerman, and Jung-Ja P. Kim. (2004). Crystal Structures of Human Glutaryl-CoADehydrogenase with and without an Alternate Substrate: Structural Bases of Dehydrogenation and Decarboxylation Reactions. Biochemistry, 43, 9674-9684. Thorpe Colin, Junc-ja P. Kimt. (1995). Structure and mechanism of action of the Acyl-CoAdehydrogenases. FASEB Journal. Vol 9, 718-725. Dommes Veronika S, Wolf-H. Kunau. (1984). Purification and Propertieso f Acyl Coenzyme A Dehydrogenasesfrom Bovine Liver.The Journal Of Biological Chemistry, 259, 1789 – 1797. Rhead William J, Brad A. Amendt, Kathie S. Fritchman, Sara J. Felts. (1983), DicarboxylicAciduria: Deficient [1-{14}C]OctanoateOxidation and Medium-Chain Acyl-CoADehydrogenase in Fibroblasts. Science, Vol. 221, 73-75. DaftarPustaka

  18. TerimaKasih

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