Term
Treatment for PDH deficiency |
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Definition
- Ketogenic diet- high fat, low carbs
- Dicholoacetate- blocks enzyme that phosphorylates PDH, thus inactivating it. Therefore, DCA keeps PDH active
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Term
Dietary thiamine deficiency |
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Definition
- Cofactor for E1 of PDH (which decarboxylates pyruvate)
- Can lead to Berberi or Wernicke-Korsakoff syndrome (in alcoholics)
- Neurological and cardiovascular disorder often seen in malnurished individuals
- *Unable to oxidize pyruvate
- Organs, like brain, that heavily depend on aerobic oxidation of glucose are affected
- *Elevated levels of pyruvate, alanine, and lactate often seen in blood
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Term
Neuropsychiatric Syndromes resulting form thiamine deficiency |
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Definition
Wernicke's encephalopathy
- Triad of opthalmoplegia:
- Weakness or paralysis or eye
- Ataxia- unbalanved
- Sudden confusion
- *Nystagmus- rapid involuntary eye movements
Korsakoff psychosis
- Persistent deficits in learning and memory
- Amnesia: Anterograde (rapidly forget new info) and Retrograde (forget old info)
- Confabulation- unable to recall what really happened, while making up plausible stories, without being aware of doing so
- Wernicke-Korsakoff syndrome when occurs in conjection with Wernicke's encephalopathy
- Hemorrhages are classic signs
Medical emergencies
- *IV thiamine must be given BEFORE IV glucose
- Also seen in extended vommitting, thiamine deficiency, and systemic diseases like HIV, cancer, uremia with hemodialysis
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Term
Allosteric and covalent regulation of PDH complex |
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Definition
Turned off when energy is high and turned on when energy is low- you don't need more energy if you have a lot
Allosteric
- Activation: low energy molecules
- Long chain FA, NADH, acetyl-COA, ATP
- Inhibition: high energy molecules
Phosphorylation
- Protein kinase- inactivates E1
- Inhibited by high levels of ATP
- Phosphoprotein phasphatase- active E1
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