In this course, some general features of mitochondria have been described. In addition, new articles related to mitochondria have been discussed. you can also find some information about mitophagy and pharmaceutical approaches in order to cure fatal diseases such as cancer.

1. Arad Boustan
Under supervision of Dr. Jalili
1

2. MITOCHONDRION
Mitos= thread Chondrion= "granule" or "grain-
like“*
2

3.  Diameter: between 0.75 and 3 μm
 Circular DNA
 Contains outer and inner membrane
 Intermembrane space
 Cristae
 Matrix
 Dynamic but not always completely
3

4. 1890 Richard Altmann
Probably is the first person observed and
categorized mitochondrion as an organelle.
He called it "bioblast"
1898Carl Benda
The term "mitochondria" was coined by
Carl Benda in 1898
Richard Altman
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6. Glossary:
Swell:‫تورم‬
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7. es
What are the problems of Endosymbiosis?
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8. 8

9. MitoFusiN
9Molecular Cell Biology 8th 2016

10. MITOCHONDRIA-ASSOCIATED
ENDOPLASMIC RETICULUM
MEMBRANE(MAM)
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11. Interaction of mitochondria with the
endoplasmic reticulum. (a) Fluorescence light
microscopy shows that tubules of the ER
(green) wrap around parts of the
mitochondrial network (red) in mammalian
cells. the mitochondria then divide at the
contact sites. after contact is established,
fission occurs within less than a minute, as
indicated by time-lapse microscopy. (B)
Schematic drawing of an ER tubule wrapped
around part of the mitochondrial reticulum.
It is thought that ER–mitochondrial contacts
also mediate the exchange of lipids between
the two membrane systems.
11Molecular Biology of the Cell ALBERTS 2015

12.  Mitochondrial shape and dynamics
 PINK1/Parkin-dependent mitophagy (initiated at MAMs)
 Calcium transport into the mitochondria Calcium homeostasis and calcium-mediated
signaling
 Glucose and energy metabolism
 Mitochondrial import from the ER of lipids, including phosphatidylserine and possibly
cholesterol
 Mitochondrial biosynthesis of lipids, including phosphatidylethanolamine and steroid
hormones
 Responses to stress Cell survival via regulated cell death
 Inflammatory responses via the inflammasome and innate immune responses
 Pathways implicated in viral infections (cytomegalovirus, hepatitis C virus)
Neurodegenerative pathology (Alzheimer’s and Parkinson’s diseases)
12*Molecular Cell Biology 8th 2016

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14. 14

15. It is now clear that
mitochondria participate in
nearly all aspects of cell
function
15

16. 16Molecular Biology of the Cell ALBERTS 2015

17. 17Molecular Biology of the Cell ALBERTS 2015

18. 18

19. 19https://journals.plos.org/plospathogens/article/figure?id=10.1371/journal.ppat.1000018.g003

20. 20
Massimo Bonora and Paolo Pinton ; Mitochondria DNA Keeps You Young DOI 10.1038/s41419-
018-1045-4

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22.  Base excision repair
 Double strand break repair
 Direct reversal
 Mismatch repair
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25. Mitochondria, the organelles that power cells, harbor their own DNA, and scientists
long thought that only mothers could pass this genetic material on to their children.
But a new study suggests dads may not be the deadbeats we thought they were,
PBS’s NOVA reports. By sequencing mitochondrial DNA from individuals with
mysterious symptoms and their relatives, scientists discovered evidence
for paternal inheritance in 17 people from three different families, they reveal this
week in the Proceedings of the National Academy of Sciences. Father-to-offspring
transmission—which researchers have seen before in sheep, flies, and even mice—is
probably rare in humans, but the new work suggests more of us may be carrying a
mitochondrial legacy from our dads than we realized.
25
Proceedings of the National Academy of Sciences Nov 2018, 201810946; DOI:
10.1073/pnas.1810946115

26. WHY?
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27. Approximately 1000 proteins encoded
by nuclear genes (more than 95%
of mitochondrial proteins) are
synthesized on free cytosolic ribosomes
and
imported into mitochondria as
completed polypeptide chains.
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29. 29

30.  Large Scale mtDNA rearrangement
 Kearns-Sayre syndrome
 Pearson Syndrome
 Progressive external ophthalmoplegia
 Point Mutation mtDNA
MELAS Syndrome
 Leber's hereditary optic neuropathy
myoclonic epilepsy with ragged red fibers (MERRF
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31.  Nuclear Genes
Wilson's disease
Friedreich's ataxia
hereditary spastic paraplegia
 A variety of disorders can be caused by nuclear mutations of oxidative
phosphorylation enzymes such as coenzyme Q10deficiency and Barth syndrome
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