Koc - Mitochondrial Biogenesis and Disease

Question 1

How many proteins does the Mt genome code?

How many tRNAs are required?

Answer 1

13 essential proteins

22 tRNAs

Question 2

What is the nature of Mt ribosomes?

Answer 2

Very similar to backteria, same translation factors

Question 3

What is supporting evidence of mitochondrial endosympbiotic theory?

Answer 3

1. Separate genome
2. Robisomes-protein synth sensitive to antibiotics
3. N-formyl methioning initial amino acid

Question 4

What replicates mtDNA?

Answer 4

DNA pol-Y (gamma)

Question 5

MERRF

Answer 5

Mutation in mitochondria tRNA

Ragged red fibers

Question 6

LHON

(Leber Hereditary Optic Neuropathy)

Answer 6

NADH Dehydrogenases

Vision loss (inherited)

Question 7

MELAS

(Mt Encephalomyopathy, lactic acidosis, and stroke-like episodes)

Answer 7

mt tRNA

brain, nervous system, muscles

Question 8

Diagnostic tests for mitochondrial disease

Answer 8

Family history, muscle biopsies, blood tests, genetic testing

Question 9

Treatment for mitochondrial disease

Answer 9

Creatine, Co-Q10 supp, keto-diet

Nucleside Bypass Therapy (Charlie Gard)

Three Parent Babies

Question 10

HIF1A

Answer 10

Causes blood vessel growth that feed tumors, activated by ROS

Question 11

Diabetes and mitochondria

Answer 11

High caloric intake can cause dysregulation of metabolic enzymes, and mutations; over production of ROS

disruption of insulin signaling

Question 12

What can reduced mitochondrial biogenesis result in?

Answer 12

Large adipocytes, genetic predisposition to obesity

Question 13

Doxorubicin

Answer 13

induces ROS production by binding to cardiolipin and increasing free iron accumulation

drives APOPTOSIS

Question 14

Trastuzumab

Answer 14

Blocks Her2 signaling, disrupts cardioprotective machinery

APOPTOSIS

Question 15

Zidovudin (Azidothymidine, AZT)

Answer 15

mtDNA pol-y (gamma)

inhibits transport of ATP and replication of mitochondria