The Structure and Functions of Mitochondria

Casp9
APAF1 Mitochondrial Control of Apoptosis
Pi Cyt-C Signaling Pathway
ATP Apoptosis is a process of programmed cell death that
ADP serves as a major mechanism for the precise regulation
(ARG54058)
of cell numbers. In mammals, activation of caspases is
HK (ARG20178)
C under tight control of the BCL family proteins,
VDA Cyt
-C
(AR tB including BCL2 and BAX. These proteins mainly act by
ANT
(ARG65581) G20
006
) id
(ARG54380) regulating the release of caspase activators such as
M ARG655
(
cytochrome C and AIF from the inner matrix of
ADP ATP TC 87) mitochondria.
NADH FAD H2
NAD +

coenzyme A FADH2

acetyl CoA
TCA CO2
(ARG
6352
CLPP6) Proteolysis
Biogenesis of Mitochondria: Protein
Cycle Unfolded
protein
Transport and Assembly
(ARG54173)
ADP RO HSP60 6250
7) Approximately 1500 protein are present in the
ATP S (ARG
0 mitochondria. The import of mitochondrial proteins
HSP7 Proteolytic
(ARG62603)
Folding
(ARG54100)
Prohibitin assembly synthesized in the cytoplasm and their direction to the
TXNRD2
ROS
MPP88) processing
655
correct compartments involves multiple pathways and
O2 H2O UCP (ARG64241) (ARG
NADH NAD++H+ CV macromolecular machinery. Preproteins synthesized in
CIV SOD Tim
FADH2 FAD +2H CIII ROS the cytoplasm translocate to mitochondrial matrix
CI + +

through the Translocase of Outer Membrane (TOM)

CII ROS 40 9)
ROS
Tom (ARG2022 a n d Tr a n s l o c a s e o f I n n e r M e m b r a n e ( T I M )
ROS
macrocomplexes. HSP70 and HSP60 chaperones
ATP Synthase Fatty Acid Biosynthesis:
UCP1 (ARG63944)
further process the preproteins by folding and
COX IV
Core subunits: Accessory subunits: Cyt-C
(ARG54003) UCP2 (ARG64193) assembly. The unfolded protein will then be cleaved
NDUFS1 (ARG65573) NDUFA13 (ARG65563) Reductase
SDHA UCP3 (ARG65580) and lysed by ATP-dependent mitochondrial
NDUFS2 (ARG65574) NDUFA2 (ARG65564)
NDUFS3 (ARG65575) NDUFA4 (ARG65565)
SDHB (ARG65579) peptidase CLPP.
SDHC
NDUFS7 (ARG65316) NDUFA5 (ARG65566)
SDHD
NDUFS8 (ARG65578) NDUFA6 (ARG65567)
NDUFA8 (ARG65568)
Accessory factors: NDUFA9 (ARG65569)
NDUFAF1 (ARG65570) NDUFS4 (ARG65576)
NDUFAF2 (ARG65571) NDUFS5 (ARG65577)
NDUFAF4 (ARG65572) NDUFS6 (ARG65311)