Lecture 3

Question 1

What part of the mitochondria is responsible for oxidative phosphorylation?

Answer 1

Inner membrance

Question 2

What part of the mitochondria is responsible for the electron transport chain?

Answer 2

Intermembrane space

Question 3

What part of the mitochondria is responsible for the TCA cycle and fatty-acid oxidation?

Answer 3

Matrix

Question 4

How many transcriptional starting sites are there for mitochondrial DNA?

Answer 4

3

Question 5

What is the total number of genes encoded by mitochondrial DNA?

Answer 5

37

Question 6

How many protein complexes in the inner mitochondrial membrane are involved with the ETC?

Answer 6

4

Question 7

What are the two origin sites for mitochondrial DNA transcription?

Answer 7

OH and OL

Question 8

What is mitochondrial mosaicism?

Answer 8

Different cells that derive from a same parent cell contain different (mutant or nonmutant) mitochondria

Question 9

What is MERRF?

Answer 9

Myoclonic Epilepsy associated with Ragged Red Fibers

Question 10

What is MELAS?

Answer 10

Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like episodes)

Question 11

What is LHON?

Answer 11

Leber Hereditary Optic Neuropathy

Question 12

A-to-G mutation at nucleotide 8344 results in which disease?

Answer 12

MERRF

Question 13

A-to-G mutation at nucleotide 3243 or G-to-A mutation at nucleotide 4332 results in which disease?

Answer 13

MELAS

Question 14

G-to-A mutation at nucleotide 11778 results in which disease?

Answer 14

LHON

Question 15

What mitochondrial diseases are caused by a deletion mutation in mtDNA?

Answer 15

KSS (Kearns-Sayre syndrome), Pearson syndrome, & CPEO (Chronic Progressive Opthalmoplegia)

Question 16

Name a mitochondrial disease caused by a mutation in a nuclear gene?

Answer 16

Alpers/Ataxia (mutations in POLG)