febrina meutiawati 10507039
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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. - PowerPoint PPT PresentationTRANSCRIPT
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Febrina Meutiawati 10507039
Presentasi EnzimologiAcyl-CoA Dehydrogenase
(ACD)
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OKSIDASI-Β ASAM LEMAK
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ACYL-COA DEHYDROGENASEMerupakan Flavoenzim yang
mengkatalisis reaksi α,β-dehidrogenasi turunan asil-KoA
Terdapat 9 member dalam keluarga asil-KoA dehidrogenase, berperan dalam oksidasi asam lemak dan asam amino
Oksidasi asam lemak : SCAD, MCAD, LCAD, VLCADOksidasi asam amino : IVD, S/BCAD, GCD
MCAD (Medium Chain Acyl-CoA Dehydrogenase)
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TATA NAMA MENURUT NC-IUBMB
Termasuk ke dalam kelas enzim oksidoreduktase
SCAD (EC.1.3.99.2)MCAD (EC.1.3.99.3)IVD (EC.1.3.99.10)
LCAD (EC.1.3.99.13)
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ISOLASI DAN PEMURNIAN Acyl-CoA dehydrogenase diisolasi dari
bouvine liver mitokondria menggunakan metode acetone dry powder.
Pemurnian dilakukan denganPresipitasi Amonium SulfatGel FiltrasiKolom kromatografi Penukar Ion
DEAE-selulosaElektroforesis preparatif
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STRUKTUR 3D MCAD
Homotetramer dimer dari
dimer d = 90ÅMemiliki sisi
pengikatan untuk FAD dan asil-KoA
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STRUKTUR PITA GCD (GLUTARYL-COA DEHYDROGENASE)
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PERBEDAAN ACD OKSIDASI ASAM LEMAK DENGAN ACD OKSIDASI ASAM AMINO
Oksidasi Asam Lemak Oksidasi Asam Amino
Dehidrogenasi Dehidrogenasi dan dekarboksilasiResidu asam amino pada loop antara rantai β-sheet 4 dan 5 lebih banyak
Residu asam amino pada loop antara rantai β-sheet 4 dan 5 lebih sedikit
Residu katalitik E376 Residu katalitik E370
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MEKANISME REAKSI
pH 7,6 1˚C
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TRANSFER ELEKTRON
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KINETIKA
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MCAD
LCAD
hexadecanoyl-CoAall-cis-4,7,10,13-hexdecanoyl-Co
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INHIBISI IN VITRO SUBSRAT PALMITOYL-COA
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INHIBISISUBSRAT BUTYRYL-COA
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INHIBISISUBSRAT ISOVALERYL-COA
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DEFISIENSI ACD Dicarboxylic aciduria merupakan penyakit
yang disebabkan adanya gangguan metabolisme, yaitu β-oksidasi asam lemak C6-C10. Ditunjukkan dengan adanya episodic hypoglycemia yang tidak diikuti dengan ketosis.
Ada peningkatan jumlah C6-C10 ω-asam dikarboksilat dalam urin.
Adanya mutasi pada rantai GCD dimana LYS304 diganti oleh residu glutamat menghasilkan kekacauan metabolisme.
Adanya mutasi pada terminal karboksil pada GCD menyebabkan glutaric acidemia tipe 1.
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DAFTAR PUSTAKA Fu Zhuji, Ming Wang, Rosemary Paschke, K. Sudhindra Rao, Frank E. Frerman, and Jung-
Ja P. Kim. (2004). Crystal Structures of Human Glutaryl-CoA Dehydrogenase 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-CoA dehydrogenases. FASEB Journal. Vol 9, 718-725.
Dommes Veronika S, Wolf-H. Kunau. (1984). Purification and Propertieso f Acyl Coenzyme A Dehydrogenases from Bovine Liver. The Journal Of Biological Chemistry,
259, 1789 – 1797. Rhead William J, Brad A. Amendt, Kathie S. Fritchman, Sara J. Felts. (1983), Dicarboxylic
Aciduria: Deficient [1-{14}C]Octanoate Oxidation and Medium-Chain Acyl-CoADehydrogenase in Fibroblasts. Science, Vol. 221, 73-75.
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TERIMA KASIH