Download - MED LEZ 23 NUCLEOTIDI
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METABOLISMO DEI NUCLEOTIDI
Aspetti clinici connessi
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DIGESTIONE NUCLEOTIDI
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SINTESI EX-NOVO NUCLEOTIDI PURINICI
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5-FOSFORIBOSIL-1-PIROFOSFATO PRPP
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KREBS
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SINTESI NUCLEOTIDI PURINICI
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REGOLAZIONE
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VIE DI RECUPERO
200x
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http://www.lesch-nyhan.org/it/
I pazienti con la malattia di Lesch-Nyhan hanno quasi sempre problemi comportamentali. Tentare di colpire gli altri, sputare sulle persone o usare un linguaggio volgare sono alcuni dei loro cattivi comportamenti. Questi comportamenti possono sembrare atti di aggressione verso gli altri, ma non lo sono. Le persone con la malattia di Lesch-Nyhan non sono aggressive, molti riferiscono di agire sotto impulso e di non potersi controllare, e spesso si scusano dopo aver fatto qualcosa di brutto. Nonostante il rimorso per questi cattivi comportamenti, spesso gli impulsi sono irresistibili e incontrollabili.
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SINTESI EX-NOVO NUCLEOTIDI PIRIMIDINICI
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CAD
UTP
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UTP CTP
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REGOLAZIONE
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SINTESI NUCLEOTIDI PIRIMIDINICI
N5 N10 metilenTHF
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DEOSSIRIBONUCLEOTIDI
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DEOSSIRIBONUCLEOTIDI
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Vie di Recupero
CITOSINATIMIDINA PRPPURACILE
NUCLEOSIDE- 5Pi + PPi
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DEGRADAZIONE BASI AZOTATE
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ACIDO URICO
POCO SOLUBILE (6.5 mg/100 ml)
Uomo 4-7 mg/100 ml Donna 3-6mg/100 ml
GOTTA PRIMARIA (genetica)
GOTTA SECONDARIA (leucemie)
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CATABOLISMO PIRIMIDINE
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DIFFERENZE NELLE 2 SINTESI
PURINE PIRIMIDINE1.ANELLO COSTRUITO 1. PRIMA COSTRUZIONE
SUL RIBOSIO ATTIVATO ANELLO E POI LEGAME
(PRPP) CON PRPP
2. ESSENZIALE 2. NON ESSENZIALE ACIDO TETRAFOLICO ACIDO TETRAFOLICO
3. VIA RAMIFICATA 3. VIA UNICA
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DIFFERENZE nellaDEGRADAZIONE
PURINE PIRIMIDINEIL PRODOTTO E’ I PRODOTTI
L’ACIDO URICO SONO CO2
(poco solubile) NH3 (solubili)
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ANTIMETABOLITI
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