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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

PENTOSA PHOSPAT PATHWAY (G6PD DEFISIENCY)

OLEH:

ANIS MURNIATI

126070100111028






Defisiensi g6pd
DEFISIENSI G6PD

  • X-linked recessive hereditary disseasecharacterised by abnormally low levels of G6PD

  • It caused by mutation in Xq28, environment triggers: malaria

  • Anemia hemolitic,


Hubungan eritrosit dan ppp
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



Kesimpulan
KESIMPULAN

  • Lintasanpentosafosfatmerupakanjaluralternatifuntukmetabolismeglukosa.

  • Lintasaninimempunyaiduafungsiutama , yaitu : produksi NADPH untuksintesisreduktifsepertibiosintesisasamlemakserta steroid danproduksiresiduribosauntukbiosintesisnukleotidasertaasamnukleat.

  • LintasanPentosaFosfattidakmenghasilkan ATP.

  • Faseoksidatifmenghasilkan NADPH danfasenonoksidatifmenghasilkanprekursor.

  • Defisiensi G6PD, stresoksidatifdapatmenyebabkandistructionprematur RBC karenakurangnyaenzim yang disebut glutathione menyebabkananemia hemolytic.



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