Relational Network of the Branched-Chain Alpha-Keto Acid Dehydrogenase Complex
The relational network of the BCKDH is below. Further below in this article, we will get into the “Maple Syrup Urine Disease” lower on down, so do not be alarmed by this part of the chart just yet.
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This is an system of enzymes your body uses to break the BCAAs down.
The enzymes, or cofactors, required for this complex are as follows:
Branched-Chain Alpha-Keto Acid Dehydrogenase Complex Enzymes
- Thiamine diphosphate
- FAD
- NAD+
- Lipoate
- Coenzyme A
Note that a cofactor (or in this case, enzyme) is merely a chemical compound that is NOT a protein, but is bound to a protein, yet is required for biological processes to function. Consider it a “helper” compound to make the necessary biological reactions occur.
Branched-Chain Alpha-Keto Acid Dehydrogenase Complex Deficiency – “Maple Syrup Urine Disease”
Although extremely rare, if newborn babies do not have this enzyme complex, their bodies are unable to degrade the leucine, isoleucine, and valine in the branched-chains, which will lead to “maple syrup urine disease”, a metabolic disorder that is noticed during early infancy due to the sweet odor of urine.
For all other humans, BCAAs break down quite easily and are an incredible way of preventing catabolization (muscle breakdown) during exercise or fasting (or during “fasted” exercise).The Gory Scientific Details of the Branched-Chain Alpha-Keto Acid Dehydrogenase Complex
The full nucleotide sequence to encode the the human dihydrolipoyl transacylase (E2) CDA is accomplished by using the (RACE) procedure. This cDNA is 3535 nucleotides long, and its coding region is 1446bp, while the 3′-non-coding region is 2074bp. This non-coding-region has 3 Alu sequences in repetition as well as two transcription termination sites.
Did you know… BCAAs are the ONE supplement for which there is no true food substitute when looking for fast-acting protein synthesis to prevent muscle breakdown. This is due to the fact that these supplements are not “peptide-bonded”, as is the case in whey protein powders, so they do not require digestion mechanisms to break them down.
