Qcm Bioch 3 Flashcards

1
Q

Leucinose caractéristique

A

Accumulation d’acide alpha cétonique dans les urines

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2
Q

Z tyro a partir de phe nécessite co fact enzymatique

A

Tetrahydrobiopterine

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3
Q

Trouble du metabo des biopétrine

A

Ø Z de tyro

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4
Q

Régénération BH4 a Partir de BH2

A

Dihydrobiopterine redu

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5
Q

Pyruvate -> lactate

A

DHGase -> NADH,H+

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6
Q

Phenycetonurie regle spé

A

Ø viande / oeuf

O sucre / graisse

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7
Q

Régime phe dosage

A

2 fois/ semaines par prélèvement sanguin

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8
Q

Tyrosine AA

A

Glucoformateur et cetogene

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9
Q

Hypertyrosinemie provoque

A

Atteinte oculaire / cutané

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10
Q

Tyrosinemie de type 1 : régime

A

Stricte a vie

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11
Q

Phe ce ton regime

A

Jusqu’à 12 an strié

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12
Q

Tyrosinemie de type 1 digno biologique

A

Succinyl acétone dans les urines

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13
Q

Metabo homocysteine : coenz

A

B6
B9
B12
Betaine

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14
Q

Met :

A

Donneur de methyl

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15
Q

Met activer

A

SAM

S adenosyl met

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16
Q

Met -> SAM -> SAH ->

A

Homocysteine

S adenosyl homocysteine

17
Q

Accumulation homocysteine

A

Thromobooser

18
Q

Homocystinurie epidemio

A

1/ 185000
Atteinte squelettique : syndrome MArfanoide /ostéoporose/hyperlaxité ligamentaire
Atteinte oculaire : luxation du cristallin/ myopie importante/ cataracte

19
Q

Homocystinurie pic

A

HomocystINE :nouveau

20
Q

Régime homocysteine

A

Hypoprot strict a vie

21
Q

Catabolisme : valine

A

Succinyl coa

22
Q

Catabolisme leucine

A

Acetoacetate / acetylcoa

23
Q

Catabolisme ÎLE

A

Propionyl coa

24
Q

Valine AA

A

Glucoformateur

25
Q

Leucine AA

A

Cétogène

26
Q

Leucinose du

A

Déficit DHGASE en acide alpha cétonique

27
Q

Leucinose epidemio

A

1/150 000

Troule neurologique

28
Q

Leucinose diagnose

A

Chromatographie AA plasmatique

29
Q

Régime leucinose

A

Øaliment trop riche en prot : viande / lait / céréale