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PENTOSA PHOSPAT PATHWAY (G6PD DEFISIENCY)

PENTOSA PHOSPAT PATHWAY (G6PD DEFISIENCY). OLEH: ANIS MURNIATI 126070100111028. INTRODUCTION. REAKSI PPP. FASE OKSIDATIF. FASE NONOKSIDATIF. DEFISIENSI G6PD. X-linked recessive hereditary dissease characterised by abnormally low levels of G6PD

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PENTOSA PHOSPAT PATHWAY (G6PD DEFISIENCY)

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  1. PENTOSA PHOSPAT PATHWAY (G6PD DEFISIENCY) OLEH: ANIS MURNIATI 126070100111028

  2. INTRODUCTION

  3. REAKSI PPP

  4. FASE OKSIDATIF

  5. FASE NONOKSIDATIF

  6. DEFISIENSI G6PD • X-linked recessive hereditary disseasecharacterised by abnormally low levels of G6PD • It caused by mutation in Xq28, environment triggers: malaria • Anemia hemolitic,

  7. HUBUNGAN ERITROSIT DAN PPP ketidakmampuanmempertahankanreduksi glutathione diseldarahmerah meningkatkanakumulasiperioksida, terutama H2O2, melemahnya hemoglobin padamethemoglobinjugapadadindingsel Glutathione perioksidaberpindahkembalimelaluiaksiperioksida glutathione. H2O2 meningkat,Stressoksidatifmeningkat Membran SDM rentandanmudahpecah Hemolisis Glikolisismenyediakan ATP untukmemompa ion dimembrandan NADH untukoksidasikembalimethemoglobin. Pentosa Phosphate Patwaymensuplaiseldarahmerahdengan NADPH untukmempertahankanfasereduksidari glutathione

  8. PATOFISIOLOGI

  9. KESIMPULAN • Lintasanpentosafosfatmerupakanjaluralternatifuntukmetabolismeglukosa. • Lintasaninimempunyaiduafungsiutama , yaitu : produksi NADPH untuksintesisreduktifsepertibiosintesisasamlemakserta steroid danproduksiresiduribosauntukbiosintesisnukleotidasertaasamnukleat. • LintasanPentosaFosfattidakmenghasilkan ATP. • Faseoksidatifmenghasilkan NADPH danfasenonoksidatifmenghasilkanprekursor. • Defisiensi G6PD, stresoksidatifdapatmenyebabkandistructionprematur RBC karenakurangnyaenzim yang disebut glutathione menyebabkananemia hemolytic.

  10. THANK’S

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