Glycogen storage disease type VII or Tauri’s disease, results from deficiency of the enzyme phosphofructokinase-1 (PFK-1) in muscle. The metabolic outcomes in muscle cells are likely to include blocked glycolysis and:Group of answer choiceslow [ATP], accumulation of glycogen, downregulation of oxidative phosphorylation.low [ATP], accumulation of glycogen, upregulation of oxidative phosphorylation.high [ATP], depletion of glycogen, upregulation of oxidative phosphorylation.high [ATP], accumulation of glycogen, downregulation of oxidative phosphorylation.low [ATP], depletion of glycogen, upregulation of oxidative phosphorylation.high [ATP], depletion of glycogen, downregulation of oxidative phosphorylation.low [ATP], depletion of glycogen, downregulation of oxidative phosphorylation.high [ATP], accumulation of glycogen, upregulation of oxidative phosphorylation.
Question
Glycogen storage disease type VII or Tauri’s disease, results from deficiency of the enzyme phosphofructokinase-1 (PFK-1) in muscle. The metabolic outcomes in muscle cells are likely to include blocked glycolysis and:Group of answer choiceslow [ATP], accumulation of glycogen, downregulation of oxidative phosphorylation.low [ATP], accumulation of glycogen, upregulation of oxidative phosphorylation.high [ATP], depletion of glycogen, upregulation of oxidative phosphorylation.high [ATP], accumulation of glycogen, downregulation of oxidative phosphorylation.low [ATP], depletion of glycogen, upregulation of oxidative phosphorylation.high [ATP], depletion of glycogen, downregulation of oxidative phosphorylation.low [ATP], depletion of glycogen, downregulation of oxidative phosphorylation.high [ATP], accumulation of glycogen, upregulation of oxidative phosphorylation.
Solution
The correct answer is: low [ATP], accumulation of glycogen, upregulation of oxidative phosphorylation.
Here's why:
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Glycolysis is the process by which glucose is broken down to produce ATP, the main energy currency of the cell. In Glycogen storage disease type VII, the enzyme phosphofructokinase-1 (PFK-1) is deficient. PFK-1 is crucial for glycolysis, so its deficiency would block glycolysis, leading to low ATP levels.
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Glycogen is a storage form of glucose. If glycolysis is blocked, glucose would not be used up and would instead accumulate as glycogen.
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Oxidative phosphorylation is another process by which cells produce ATP. If glycolysis is blocked, the cell would try to compensate by upregulating oxidative phosphorylation to produce more ATP.
Similar Questions
n muscle tissue, adrenaline signalling results inGroup of answer choicesDephosphorylation of the bifunctional PFK-2/FBPase-2 enzyme which increases glycolysis.Phosphorylation of glycogen phosphorylase which decreases glycogenolysis.Increased levels of fructose-1,6-bisphosphate production, which allosterically upregulates pyruvate kinase.The R state of phosphofructokinase-1 is not stabilised which decreases glycolysis.
Contrairement au foie, le muscle ne contribue pas directement a la régulation de la glycémie carADépourvu de glycogène phosphorylaseBDépourvu de glucokinaseCDépourvu de glucose-6-phosphataseDDépourvu d'enzyme débranchanteEPauvre en glycogène
A propos de la régulation de la glycogénolyse, retenir la bonne réponse :ADans le muscle, L'ATP et le G6P sont des inhibiteurs allostériques de la glycogène phosphorylaseBLa forme non phosphorylée tendue de la glycogène phosphorylase est la forme activeCL'état R relâché de la glycogène phosphorylase est phosphorylé et très peu actifDLe glucagon inhibe la glycogène phosphorylase kinaseEDans le foie, Le glucose est un activateur allostérique de la glycogène phosphorylase
Concernant la régulation du métabolisme du glycogene :1/ l'inactivation de la glycogène phosphorylase est catalysée par une kinase2/Le glucagon active la glycogénolyse par activation de l'adénylate cyclase au niveau du foie3/L'ATP et le G6P sont des activateurs allostériques de la glycogène phosphorylase4/La forme phosphorylée de la glycogène synthase est active5/En période post-prandiale, l'insuline accélère la glycogénogenèseA2,5B2,3,4C4,5D1,2,2E1,4
Gluconeogenesis, must bypass irreversiblereactions of glycolysis, except:a. Hexokinaseb. Phosphohexose isomerasec. Pyruvate kinased. Phosphofructokinase
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