MTHFR part 6: Folate Cycle continues… Urea Cycle

Please note: The content of this post is my own, unless the technical terms is too hard to explain, then the content is copy and pasted. I am not a medical professional and thus the post is my point of view. But the content is science-based and credible. Just because it is a new science, does not necessarily make it quackery. 

Urea Cycle – The Waste Facility

The the main and most important purpose of the Urea Cycle, also know as the Krebs-Henselein cycle is to remove ammonia from the body. the process mostly takes part in the liver and the kidneys. Ammonia is very toxic and even the smallest amount can cause serious damage.

This is also your CBS part of the MTHFR.

Sticking to the analogy of this cycle being like a waste management facility, power is received from the power plant (mitochondria), the previous cycle BH4 share their facility for the urea cycle to clean out the ammonia. This generous supply by BH4 also allows NOS (Waste Management Super) to make nitric oxide. Nitric Oxide maintains blood vessels throughout the whole body preventing plaque to build up which can lead to strokes and other heart conditions.

Looking at Alex’s results on CBS:

Now, if your CBS is not working properly and the “waste” spills over to form free radical of which Super Oxide and Peroxynitrate is the most destructive to healthy cells. This can cause the following defects:

  • N-Acetylglutamate synthase deficiency (Hyperammonemia, type 111 or NAGS deficiency: inherit disorder that causes ammonia to build up in the Blood, link)
  • Carbamoyl phosphate synthetase deficiency (congenital Hyperammonemia, type 1: link)
  • Ornithine transcarbamoylase deficiency (OTC deficieny: link)
  • Citrullinemia (Deficiency of argininosuccinic acid synthase: link)
  • Argininosuccinic aciduria (Deficiency of argininosuccinic acid lyase: link)
  • Argininemia (Deficiency of arginase: link)
  • Hyperornithinemia, hyperammonemia, homocitrullinuria syndrome (Deficiency of the mitochondrial ornithine transporter: link)

Most urea cycle disorders are associated with hyperammonemia, however argininemia and some forms of argininosuccinic aciduria do not present with elevated ammonia.

One of the most common conditions for a defective urea cycle is Gout. The build-up of uric acid causes inflammation and pain in the joint tissue. Uric acid is produced when the body breaks down a chemical called purine. Purine occurs naturally in your body, but it’s also found in certain foods. Uric acid is eliminated from the body in urine.

So, what food can you eat to lower purines and ammonia in your body:

Lastly the Citric Acid Cycle….

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