porfiri nas
DESCRIPTION
presentación de porfirinaTRANSCRIPT
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Dra. Carmen Aída Martínez
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Grupo Hem
CH
CH
HC
HC
Anillo pirrol
I
II
III
IV
Porfirina
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Formación del anillo pirrólico
Condensación de 4 grupos pirrólicos para dar un tetrapirrol cíclico
Modificación de la cadena lateral y oxidación del anillo (porfirina)
Adición del metal
Síntesis del grupo Hem
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Síntesis del ALA
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Porfobilinógeno hidroximetilbilano
Uroporfirinógeno I
sintasa
Uroporfirinógeno III
cosintasa
Uroporfirinógeno III
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Acetato
-CH2COOH
Propionato
-CH2CH2COOH
Metilo
-CH3
Vinilo
-CH = CH2
Tipo I y III
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Uroporfirinogeno
descarboxilasa
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Coproporfirinogeno
oxidasa
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protoporfirinogeno
oxidasa Ferroquelatasa
Fe++ 2H6H
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Sitios principales de biosíntesis del Hem:◦ Eritrocitos (85%)
◦ Hígado
Hígado: ◦ ALA sintasa
Eritrocito:◦ Ferroquelatasa
◦ uroporfobilinógeno sintasa
◦ Hemo estimula síntesis de globina
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PORFIRIAS
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Porfirias: porfiria eritropoyetica
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Metabolismo del hem
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NADPH + H
NADP
bilirrubina
Biliverdina
Hemo
CO + H2O + NADP
Fe3+
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Prehepática
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hepática
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Posthepática