Biochemistry of Tyrosinemia [TYPE 1]

NOW...
We let you guys off easy yesterday...
How about something more challenging today?
Today's post will be about the biochemistry of tyrosinemia type 1, which may require a little more of your brain juice to understand!
We took some time ourselves too heheh!

The catabolic pathways of tyrosine
 

The FAH enzyme is responsible for converting tyrosine catabolic intermediate fumarylacetoacetate into fumaric acid and acetoacetic acid.
Under normal conditions, where the FAH enzyme is present, fumarylacetoacetate can be successfully converted into fumaric acid and acetoacetic acid.
However, when the FAH enzyme is deficient, it will lead to an increase in the pathogenic fumarylacetoacetate, which will be converted into succinylacetoacetate. Succinylacetoacetate will be decarboxylated to eventually form the toxic product, succinylacetone.

Structure of succinylacetone

Accumulation of succinylacetone inside the blood can cause serious damage to organs and tissues, especially in the liver and kidneys. Inside the kidneys, succinylacetone acts as a mitochondrial toxin and blocks the substrate-level phosphorylation in Krebs cycle

THE END!
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