MODY- diabetes

[nurmd01]

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This question has been bugging me...

So...
In MODY (Maturity onset diabetes of the young), there is a mutation in glycokinase. Glycokinase is found in liver and beta cells of pancreas. (hexokinase in rest of the tissues). Glycokinase serves as a glucose sensor for pancreatic B cells. So, during hyperglycemia glucose is taken by the b-cells of pancreas and via glycokinase they are further gone thru the glycolysis pathway which in turn increases ATP causing release of insulin.
What I don't get is...
MODY is not too serious disease, it is usually detected accidentally (mild elevated blood glucose)... how is this possible considering that MODY causes glycokinase mutation which is key in insulin production? so how is insulin released when glycokinase is needed for this pathway?

 

[Apoplexy__]

Well first, MODY isn't just a glucokinase mutation -- there are other possible mutations as well. Glucokinase is just the only tested one.

Second, there are many different possible glucokinase mutations, producing glucokinase enzymes with varying affinities for glucose. I think you're assuming the mutation in question produces a knockout, where there would be absolutely no glucokinase in the cells, but I think the reality is that these possible mutations produce a spectrum of affinity for glucose.

 

[EmetyreUSMLEStep1Tutor1]

Do they ask about MODY diabetes on step 1?🙁