ADA defiency

[aspiringmd1015]

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in ada defiency for scid it mentions that its toxic to the cell bc of the toxic byproducts of the purine salvage pathway, so in the pathway adenosine normally gets demaniated to inosine, so its the adenosine which is toxic to the lymphocytes? (and inosine bc some cases of scid can also be due to nucleoside phosphorylase)

 

[dyspareunia]

ADA deficiency -> ATP and dATP buildup -> ribonucleotide reductase inhibition -> impaired DNA synthesis -> low T and B and NK cells.

PNP deficiency -> dGTP buildup -> toxic to T cells

 

[aspiringmd1015]

thanks, btw ribonucleotide reductase reduces ntp's into dtps, how is it reducing it if its removing a OH group on the 2nd carbon of ribose?

 

[SBR249]

thanks, btw ribonucleotide reductase reduces ntp's into dtps, how is it reducing it if its removing a OH group on the 2nd carbon of ribose?

Any enzyme can simultaneously catalyze the forward and reverse reactions, sometimes it's named for the forward reaction, other times, for the reverse.

 

[dyspareunia]

thanks, btw ribonucleotide reductase reduces ntp's into dtps, how is it reducing it if its removing a OH group on the 2nd carbon of ribose?

I'm pretty sure removal of an oxygen defines a reduction reaction. Don't know for sure, and don't really care tbh. We aren't studying for the MCAT here.

 

[SBR249]

I'm pretty sure removal of an oxygen defines a reduction reaction. Don't know for sure, and don't really care tbh. We aren't studying for the MCAT here.

Pretty sure you are right, totally missed that.

Source: http://chemwiki.ucdavis.edu/Analyti...ion_and_reduction_in_terms_of_oxygen_transfer