Metabolisme porfirin.ppt - Metabolisme porfirin dan pigmen...

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Metabolisme porfirin dan pigmen empedu Juliana Christyaningsih
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Pendahuluan Porfirin merupakan senyawa siklik yg terbentuk dr penggabungan 4 cincin pirol lewat jembatan metenil (-HC=). Sifat khas porfirin : terbentuk secara kompleks dg ion-ion logam yang terikat pd atom nitrogen cincin pirolnya.
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Struktur Porfirin
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Keberadaan Porfirin Senyawa porfirin yang terikat besi disebut heme pd hemoglobin Senyawa porfirin yang terikat magnesium disebut klorofil , merupakan pigmen fotosintesis pd tumbuhan. Protein mengandung heme (hemoprotein) yg tdp dlm tbh : 1. Hemoglobin, mengangkut oksigen dlm darah 2. Mioglobin, menyimpan oksigen dlm otot 3. Sitokrom C, ikut serta dlm transpor elektron 4. Sitokrom P 450 , hidroksilasi xenobiotik 5. Katalase, degradasi H 2 O 2 6. Triptofan pirolase, oksidasi Trp
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Biosintesis Heme sitosol mitokondria
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Konversi porfobilinogen menjadi uroporfirinogen 4 mol porfobilinogen Hidroksimetilbilana Uroporfirinogen I Uroporfirinogen III Uroporfirinogen I sintase Uroporfirinogen I sintase Uroporfirinogen III kosintase Siklisasi spontan Biosintesis Heme
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Biosintesis Heme
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Porfiria Merupakan suatu kelompok kelainan bawaan metabolisme akibat mutasi gen yang mengarah pada sintesis enzim pada biosintesis heme. Gejalanya : fotosensitivitas dan cacat fisik yg parah pada penderita porfiria eritropoitik kongenital
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Mutasi DNA Kelainan enzim pd sintesis heme ALA & Porfobilinogen Porfirin Heme (sel & cairan tubuh) dlm kulit & jar Tanda & gejala Oksidasi spontan porfirinogen Neuropsikiatrik jd porfirin Fotosensitivitas
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Klasifikasi Porfiria Kelompok Tipe Porfiria Enzim yg terlibat Gejala
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  • Summer '15
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