Energetica cellulare
Energia libera e mitocondri
Origine endosimbiontica di mitocondri
e plastidi
Origine endosimbiontica di mitocondri
e plastidi
Produzione aerobica e anaerobica di ATP
Il Mitocondrio
Il Mitocondrio
Il ciclo di Krebs: produzione di coenzimi ridotti
Il ciclo di Krebs: produzione di coenzimi ridotti
Trasportatori della membrana interna
Mitocondri e perossisomi
nel catabolismo degli acidi grassi
Il catabolismo del glucosio è regolato
allostericamente
La forza lavoro attraverso la membrana interna
Control
250 µM DA
Colorazione con MitoTracker Red CMXRos
La catena respiratoria
La catena respiratoria
CoQ-Citocromo c reduttasi
Citocromo c ossidasi
Sintesi di ATP
Sintesi di ATP
Trasportatori della membrana interna
Mitocondri come depositi di calcio
Local ER–mitochondria interactions. A
representation is depicted of ER–
mitochondria relationships and of some of
the proteins involved in the cross-talk
between the organelles. Ca2+ released from
the ER through Ins(1,4,5)P3Rs is taken up by
mitochondria through the MCU. Within
mitochondria, Ca2+ modulates mitochondrial
dehydrogenase activity and thus ATP
production. Moreover, mitochondrial Ca2+
uptake regulates the spatio-temporal
pattern of the cytosolic Ca2+ signal and,
therefore, many Ca2+-dependent cellular
processes. Massive and/or a prolonged
accumulation of Ca2 in the mitochondria can
lead to the opening of the PTP in the IMM
and swelling of the organelle or, acting on
mitochondria-shaping proteins [DRP-1,
hFis1, OPA1, mitofusins (MFNs)], to
mitochondrial cristae remodeling and modulation of apoptosis. The ‘in concert’ action of the two organelles in cell death is further supported by the presence
on both the ER membrane and the OMM of different components of the Blc-2 family that have either a pro- (BIK, BAX, BAK) or an anti-apoptotic role (Bcl-2,
Bcl-XL). ER and mitochondria could be linked physically at specific sites by cytoplasmic molecules (grp75, PACS-2), either directly or through proteins expressed
on the two membranes (IP3R, VDAC). Readers are referred to the text for further details. CsmtDHs, Ca2+ sensitive mitochondrial dehydrogenases; Cyt c,
cytochrome c; IMM, inner mitochondrial membrane; IP3R, inositol 1,4,5-trisphosphate receptor; OMM, outer mitochondrial membrane; MCU, mitochondrial
Ca2+ uniporter; PTP, permeability transition pore; SERCA, sarcoplasmic-endoplasmic reticulum Ca2+ ATPase; VDAC, voltage-dependent anion channel.
Mitocondri, calcio e apoptosi
Poro di transizione mitocondriale
Mitochondrial life cycle
van der Bliek A M et al. Cold Spring Harb Perspect Biol
2013;5:a011072
©2013 by Cold Spring Harbor Laboratory Press
Controllo di qualità
Kornmann B. Quality control in mitochondria: use it, break it, fix it, trash it.
F1000Prime Rep. 2014 Mar 3;6:15. eCollection 2014. Review.
PubMed PMID: 24669296; PubMed Central PMCID: PMC3944741.
Fusione e fissione
Otera H, Ishihara N, Mihara K. New insights into the function and regulation of mitochondrial
fission. Biochim Biophys Acta. 2013; 1833:1256-68. doi: 10.1016/j.bbamcr.2013.02.002.
Fusione e fissione
Mitocondri e Autofagia
Wild & Dikic, Nature Cell Biology 12, 104 - 106 (2010)
Processamento proteolitico di PINK1 nei
mitocondri polarizzati
Degradazione
PINK1 ad
opera di una
proteasi
MG132sensibile
(Jin & Youle, 2012)
29
Accumulo della forma nativa di PINK1 nei
mitocondri depolarizzati
PINK1
c
(Jin & Youle, 2012)
30
Le proteine PINK1/Parkina mediano la
mitofagia dei mitocondri danneggiati
31
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Energetica cellulare - Uninsubria