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Samenvatting Overzicht metabole pathways biochemie $7.92
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Samenvatting Overzicht metabole pathways biochemie

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Dit overzicht geeft alle metabole pathways weer ( die gezien zijn in de lessen van prof.Roosens ) Naast elke pathway worden de reacties, regulatie mechanismen en de pathologie structureel en duidelijk uitgelegd. Pathologie is heel belangrijk om te kennen, zeker voor de open vraag gedeelte van d...

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  • November 2, 2023
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Metabole Pathways

Pathway Reacties Regulatie Pathologie
Metabolisme
Weefsel
Glycolyse Globale reactie: 1e irrev stap (gluc. -> G6P) hemolytische anemie
Koolhydraat -Katabool =G6P ‘vasthouden’ in glycolyse -tgv pyruvaat kinase deficiëntie
alle weefsels -Glucose=>Pyruvaat(aeroob) of Lactaat (anaeroob) glucokinase (hepatisch!) -RBC heeft geen mitochondriën en is voor ATP productie
[glucose]↑↑↑=↑ (EC=>IC via GLUT2, vrijstellen GK van kern naar volledig afh vd glycolyse
Doel: cytoplasma) -weinig ATP => problemen toniciteit
Primair: doorlopend energie vrijstellen [insuline]↑=↑ (meest voorkomende hemolytische anemie tgv glycolyse
-ATP [F6P]↑=↓ (=>vertraagde neg FB door binding GK in kern) deficientie ,2e meest voorkomende hemolytische anemie
-NADH => OXFOR. [glucagon]↑=↓ tgv enzymatisch deficientie)
-pyruvaat => A CoA => TCA
Hexokinase (extra hepatisch!) Arseen intoxicatie
Secundair: Hyperglycemie vermijden [glucose]↑=↑ pentavalent arseen inhibeert Glyceraldehyde-3P-
-glucose afbreken [G6P]↑=↓ (=>neg FB) dehydrogenase
=> inhibitie ATP productie
Intermediairen 2e irrev stap (F6P-F1.6BP)
-glucose=>IC via GLUT of SGLT1 =glycolyse vs gluconeogenese
-10 stappen (zie metabole kaart) phosphofructokinase-1
-regulatie in 3 irrev. stappen (zie regulatie) [F2.6BP]↑*=↑(=DOMINANT!)
[AMP] ↑=↑ (=[ATP]↓)
*[insuline]↑=>[cAMP]↓=>PKA=>PFK2=kinase=>[F2.6BP]↑

[F2.6BF]↓*=↓
[ATP]↑↑↑=↓
[citrate]↑=↓
*[glucagon]↑=>[cAMP]↑ =>PKA=>PFK2=phosphatase=>[F2.6BP]↓

[fructose]↑↑↑ => F1.6BP
= bypass 2e irrev. stap!

3e irrev stap (PEP-pyruvaat)
=glycolyse vs gluconeogenese
Pyruvate kinase
[F1.6BP]↑*=↑ (FFW 2e irrev stap)
*[PFK1]=>[F1.6BP]↑

[glucagon]↑*=↓ (hepatisch!)
=>[glucagon]↑=>[cAMP]↑ =>PKA=↓

, Oxidatie pyruvaat Globale reactie: Pyruvaat => A CoA PHD defficientie
Koolhydraat -Katabool PDH-complex -tgv gelijknamige erfelijke aandoening
alle cellen met -Pyruvaat=>A CoA [pyruvate]↑=↑ -tgv alcoholmisbruik = Wernicke-Korsakoff (B1 tekort)
mitochondrien -irreversiebel! =>[PHDkinase]↓=>PHD=actief -tgv arseen intoxicatie (trivalent arseen) inhibeert E2
Doel: [Ca2+]↑=↑ (Ca2+ tgv spieractiviteit) => ernstige problemen met CSZ tgv lactaatacidose
Koppelen koolhydraatmetabolisme ad TCA (A CoA = =>[PHDphosphatase]↑=>PHD=actief
universele intermediar TCA)
[ATP]↑=↓
Intermediairen =>[PHDkinase]↑=>PHD=niet actief
-pyruvaat=>mitochondrie via transportproteine [NADH]↑=↓
-Regelenzyme=PDH-complex (zie metabole kaart) [A CoA]↑=↓
-3 coenzyme (niet op metabole kaart!): =>[PHDkinase]↑=>PHD=niet actief
E1 Pyruvate dehydrogenase: dehydrogenase =>neg FB
E2 Dihydrolipoyl transacetylase: aanhechting CoA
E3 Dihydrolipoyl dehydrogenase: oxidatie => NADH
Gluconeogenese Globale reactie: Enkel in levercellen! Biotinidase deficiëntie
Koolhydraat -Anabool [Pyruvate carboxylase]↓
levercellen -afbraak stoffen* katabole pathways => Glucose [glucagon]↑=↑ => geen of verstoorde omzetting pyruvaat =>
*glycerol, lactaat, AZ inhibeert 3e irrev. stap glycolyse en activeert gluconeogenese oxaloacetaat

Doel: De irreversiebele stappen vd glycolyse moeten gebypassed worden
-hypoglycemie vermijden=>Synthese glucose
-linear↑ productie vanaf +/- 5u vasten Bypass-1: pyruvaat => oxaloacetaat
Pyruvate carboxylase*
Intermediairen [A-CoA uit lipide afbraak]↑=↑
≠ omgekeerde glycolyse *biotine afhankelijk!
-3 irrev. stappen vd glycolyse moeten gebypassed
worden Oxaloacetaat=>phosphoenolpyruvate
phosphoenolpyruvate carboxykinase
[GTP]↑=↑ (controlestap of productie TCA of voldoende is)

Bypass-2: F1.6BP => F6P
F1.6BPase
tegengestelde regulatie glycolyse
[F2.6BF]↓=↑
[AMP]↓ =↑

[F2.6BP]↑=↓
[AMP] ↑=↓(=[ATP]↓)

Bypass-3: G6P => glucose
G6Pase (in ER!)

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