Adenosine Deaminase Deficiency (Part 1) How Does dATP Accumulate

The video explores purine metabolism, focusing on adenosine deaminase and its broad specificity. It discusses the consequences of adenosine deaminase deficiency, leading to deoxy ATP accumulation and inhibition of ribonucleotide reductase. This inhibition affects DNA synthesis and the immune response. The video explains equilibrium reactions and Le Chatelier's principle in the context of deoxy ATP production, highlighting the importance of understanding these biochemical processes.

Why is it important to maintain low levels of ribonucleotides and deoxyribonucleotides in the cell?

• To increase cellular energy

• To prevent the formation of toxic compounds

• To enhance DNA replication

• To promote protein synthesis

What is the role of adenosine deaminase in RNA modification?

• It catalyzes the deamination of adenosine residues in RNA

• It removes inosine residues from RNA

• It adds phosphate groups to RNA

• It synthesizes RNA from DNA templates

What happens when adenosine deaminase is deficient?

• Decreased cellular ATP levels

• Enhanced DNA polymerase activity

• Accumulation of deoxy ATP

• Increased synthesis of ribonucleotides

Which enzyme is responsible for converting deoxyadenosine to deoxyadenosine monophosphate?

• Deoxyadenosine kinase

• Adenosine deaminase

• Nucleoside diphosphate kinase

• Ribonucleotide reductase

What is the role of nucleoside diphosphate kinase in the pathway discussed?

• It synthesizes RNA from DNA

• It deaminates adenosine residues

• It phosphorylates deoxyadenosine diphosphate to deoxyadenosine triphosphate

• It converts deoxyadenosine to deoxyadenosine monophosphate

How does deoxy ATP accumulation affect ribonucleotide reductase?

• It activates the enzyme

• It has no effect on the enzyme

• It allosterically inhibits the enzyme

• It converts the enzyme into a different form

What is the consequence of ribonucleotide reductase inhibition?

• Increased DNA synthesis

• Decreased synthesis of deoxynucleoside diphosphates

• Enhanced immune response

• Increased cellular ATP production

Which of the following is NOT a result of adenosine deaminase deficiency?

• Impaired immune response

• Increased DNA replication

• Inhibition of ribonucleotide reductase

• Accumulation of deoxy ATP

What is the primary substrate for DNA polymerase during DNA synthesis?

• Ribonucleoside triphosphates

• Deoxynucleoside diphosphates

• Deoxynucleoside triphosphates

• Adenosine monophosphate

What is the effect of deoxy ATP on the immune response?

• It converts immune cells into a different type

• It enhances the immune response

• It has no effect on the immune response

• It impairs the immune response