Draw out The Central Energy Pathways, ADD IN the rest of the Amino Acids that can...

Draw out The Central Energy Pathways, ADD IN the rest of the Amino Acids that can be used to create intermediates of the TCA cycle, create Pyruvate, and create Acetyl CoA. Pay special attention to all of the nutrients needed for each step in the Central Energy Pathways, making sure to include all cofactors, substrate, product, coenzymes, and enzymes. 2 points

2. Compare the Pyruvate Dehydrogenase Complex to the Branched Chain Ketoacid Dehydrogenase Complex. How are they similar and different in terms of functionality and nutrient requirements). What organic acids are elevated when there is insufficient activity of the Pyruvate Dehydrogenase Complex (PDC) vs the Branched Chain Ketoacid Dehydrogenase Complex (BCKDC)? (Make sure to understand the difference between BKCDC and BCAA transaminase. These are two completely different enzymes in the BCAA degradation pathway) 1 point

3. How does biotin relate to multiple carboxylase deficiency (MCD) and what enzymes are impaired in the absence of biotin? What are the symptoms and organic acid markers of biotin deficiency? 1 point

Expert Solution

The central energy pathway which yields pyruvate,acetyl-CoA and the other intermediates that lead to these elements:

This central energy pathway includes the TCA cycle,glycolysis,Pentose-phosphate pathway and a Entner-Doudroff pathway.The Glycolysis is the conversion of glucose to pyruvate and this takes place in cytoplasm.The TCA or Krebs which is also called the citric Acid that is the complete oxidation of pyruvate to CO2 and takes place in mitochondria.Final conversion of the energy in glucose to ATP and this takes place in mitochondria.

3)The enzymes that are impaired in the absence of biotin:

The multiple carboxylase deficiencies (MCD) are genetically determined disorders of biotin metabolism, resulting in impaired activity of the four biotin dependent carboxylases.

propionyl-coenzyme A carboxylase (PCC)
3-methylcrotonyl-coenzyme A carboxylase (MCC)
pyruvate carboxylase (PC)
acetyl-coenzyme A carboxylase (ACC)

The symptoms of this biotin deficiency is dermatitis, rash and alopecia.These symptoms are seen to be observed in nervous system, skin, respiratory system

The biotinase activity in serum was normal, indicating that this was a rare case of late-onset HCS deficiency.Multiple carboxylase deficiency (MCD) is a rare inherited metabolic disease ofbiotin dependency due to deficiency of holocarboxylase synthetase (HCS) or biotinidase deficiency.

gladiator answered 1 year ago

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