Why does adenosine deaminase defiency lead to excess ATP and dATP imbalance?

[whatdidigetinto]

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the disease is discussed on page 70 in first aid.

 

[loveoforganic2]

Buildup of adenosine (due to ADA deficiency) -> dATP buildup (due to LeChatlier's secondary to adenosine accumulation) -> Inhibition of ribonucleotide reductase -> Inhibited synthesis of other dNTPs

 

[whatdidigetinto]

I lost you at le chatelier. Please elaborate more if you can.

 

[whatdidigetinto]

wait so you mean because adenine is increasing we have more ATP being formed and the ribonucleotide reductase is working on the ATP to make dATP. and then the excess dATP is inhibiting rbonucleotide reductase?

 

[loveoforganic2]

Sorry, LeChatlier's principle here just means that the build up of adenosine (a reactant in the synthesis pathway toward dATP) leads to increased production of that dATP (the product of the synthesis pathway) to reestablish equilibrium.

Your most recent post sounds like you've got the gist of it though (at least to my understanding of what's going on)

 

[whatdidigetinto]

thanks i got it now!