med lez 25 emoglobina
TRANSCRIPT
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EMOGLOBINA eMIOGLOBINA
L’artista Julian Voss-Andreae ha creato nel 2005 una scultura dal titolo Cuore d'Acciaio (Heart of Steel),
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MIO- e EMO-GLOBINA
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MIOGLOBINA
153 aa
8 segmenti
αααα elica
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GRUPPO PROSTETICO
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GRUPPO EME
Fe++ forma
6 legami di
coordinazione
His F8
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CURVA DI SATURAZIONE
Deossigenata
Mb +O2
MbO2
Ossigenata
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GLOBULI ROSSI (12-22 g/100 ml)
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EMOGLOBINA HbA
2αααα 141aa
2ββββ 146aa
4 gruppi EME
4 02
Max Perutz 1959 -1960
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• Concentrazione di O2 0,003 ml/100ml
totale 15 ml 4 sec !!!!!!
• 280 106 molecole di Emoglobina/GR
25 109 di Emoglobina --Globuli rossi totali
20 ml/100ml
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CURVA DI SATURAZIONE
pO2 presente nei tessuti periferici
pO2 polmonare
% H
bos
sige
nata
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30
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Stato T
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Stato R
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Stato RStato T
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RILASCIO OSSIGENO
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pH
Hb-H+ + 4O2 Hb4O2 + H+
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CO2
Hb-CO2 + 4O2
Hb4O2 + CO2
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BPG / DPG
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VARIANTI FISIOLOGICHE
• Hb-A 2αααα 2ββββ 90 % • Hb-A2 2αααα 2δδδδ 10 %
• Hb-E 2ξξξξ 2εεεε embrione, 4-10 settimana• Hb-F 2αααα 2γγγγ feto, 3-9 mese
Affinità elevata per O2
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VARIANTI PATOLOGICHE
• Talassemie (α o β) mancanza di una o più
catene dell’ emoglobina
• β Talassemie β/β
• β/- minor
• -/- major si forma α2 /γ2
morte prima della maturità
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• α Talassemia αα/αα
• αα/α- normali
• αα/-- normali
• α-/-- anemici γ4 e/o β4
• --/-- idropisia fetale
morte intrauterina
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VARIANTI PATOLOGICHE
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ANEMIA FALCIFORME
SUBUNITA’ Beta
GLUT(6)
VALINA
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ANEMIA FALCIFORME
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