C11 Mitocondria Dec 2014
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Transcript of C11 Mitocondria Dec 2014
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Capitolul 15MITOCONDRIILE
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Număr
1 - Ostreococcus tauri1500 - Hepatocit
15.1. Morfologie şi ultrastructură
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Localizare - în zone cu activitate intensă
Fibră musculară
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Neuroni
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Pancreas
Celulă din glanda corticosuprarenală
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Morfologie 0.5-1 μm / 1-3 μm - 10
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Foarte mobile Schimbări de formăCresc, fuzionează, se divid
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Matrice Creste Membrana internă
Membrana externă
Ultrastructură
Modelul Palade-Sjöstrand
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ultrastructura mitocondriei
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Complexul FoF1 al ATP-sintetazei Mecanismul sintezei ATP
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Miocard
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CSR
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Neuron
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Hepatocit
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Mitocondrii izolate:conformaţia condensată
conformaţiaortodoxă
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Membrana externă: → lipide : proteine = 1 : 1→ 5% colesterol→ permeabilă pentru molecule de până la 6.000 Da (porina)
15.2. Compoziţa chimică, procesele metabolice şi enzimele localizate în mitocondrii
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Membrana internă: 25% lipide; 75% proteine → 0% colesterol
→ cardiolopină (20%)(difosfatidilglicerol)
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Conţinut mare de acid linoleic C18:3Conţinut mic de acid arahidonic C20:4
→ impermeabilă→ complexe proteice transmembranare (60) – transportsubstanţe, transportul electronilor, oxidări, sinteza de ATP
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Procese metabolice localizate în mitocondrii:procese biosintetice şi ale metabolismului energetic
Procese biosintetice generale:
- biosinteza ADN,- biosinteza ARNm,- biosinteza proteinelor
Procese biosintetice specifice:
- unele etape în sinteza hormonilor steroizi în celulele din glanda corticosuprarenală
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Proteine mitocondriale sintetizate în mitocondrie
PorinaMembrana externă
Citocromul cCitocromul bSubunitatea 4 a CoQH2-citocrom c reductazei (citocromul c1)
Spaţiulintermembranar
Transportorul ADP-ATPTransportorul Fosfat-OHSubunităţile 4,5,6 şi 7 ale citocrom c oxidazeiSubunităţile 1,2,5,6,7,8 ale CoQH2-citocrom c reductazei
Membrana internă
F1-ATP-aza: Subunităţile a,b,d, gADN, ARN polimerazele mitocondrialeProteinele ribosomaleCitrat-sintetazaOrnitin-aminotransferazaAlcool-dehidrogenaza
Matrice
PROTEINALOCALIZARE
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Acizi Graşi şi glicerol
Glucozăalte glucide Aminoacizi
Fosforilareoxidativă
ADP
Acetil-CoA
I
II
III
ATP
Hans Krebs (1900-1981)
Premiul Nobel 1953
procese ale metabolismului energetic
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Localizarea enzimelor:
→ în membrana externă: monoaminoxidaza
→ în spaţiul intermembranar: adenilatkinaza
AMP + ATP → 2ADP
→ în membrana internă: lanţul respirator mitocondrialcomplexul ATP-sintetazeitransportorii ionilor
→ în matrice: PDH enzimele implicate în β-oxidare enzimele implicate în ciclul citricenzimele implicate în sinteze de acizi nucleici/proteine
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15.3. Organizarea moleculară a lanţului respirator
H2 + 1/2O2 → H2O
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+0.21CITOCROM c1 (Fe3+)CITOCROM c1 (Fe2+)
+0.12CITOCROM b (Fe3+)CITOCROM b (Fe2+)
FAD-PROTEINĂFADH2-PROTEINĂ
+0.10UBIQUINONĂUBIQUINONĂ (UQH2)
+0.82
+0.29
-0.32
E0 (V)
1/2O2+(2H+ + 2e-)H2O
CITOCROM a (Fe3+)CITOCROM a (Fe2+)
NAD+NADH + H+
FORMA OXIDATĂFORMA REDUSĂ
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Complexul I (NADH-dehidrogenaza) >600 kDa30 polipeptide diferite4proteine cu Fe-S şi un FMN
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Complexul II (succinat-dehidrogenaza)un citocrom b şi alte polipeptide
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Complexul III (complexul citocromilor bc1) 8 subunităţi citocromul b, citocromul c1, o proteină cu Fe/S
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Complexul IV (citocrom oxidaza) 400 kDacâteva subunităţi proteiceşi citocromii a1 şi a32 atomi de Cu
O2 + 4H+ + 4e– → 2H2O
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15/40NADHDH : 3 cit. bc1 : 7 cit. a1a3 : 9 cit. c : 50 UQ
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15.4. Fosforilarea oxidativă (aspecte de termodinamică)
50% → ATP50% → căldură
3 moli Pa : 1 mol O2
3ATP
2ATP
2 moli Pa : 1 mol O2
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Piruvat Sucinat Ascorbat
AmitalRotenonă Antimycina A
KCNCO
NADH Coenzima Cit.b Cit.c1 Cit.c Cit.a1a3 O2Q10
ADP+Pi ATP ADP+Pi ATP ADP+Pi ATP
Decuplanţii fosforilării oxidative: 2,4 Dinitrofenol
Inhibitorii transferului electronilor
Inhibitorii fosforilării oxidative: oligomicina
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15.5. Mecanismul fosforilării oxidative (Mitchell, 1961)
Peter Mitchell Premiul Nobel pentru chimie (1978)
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3,5 220 mV
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F13 α, 3 β, 1 δ, ε, γ
Fo (o = oligomycin) 1 a, 2 b, 10 c
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Complexul FoF1 al ATP-sintetazei Mecanismul sintezei ATP
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Paul D. Boyer
John E. Walker
Premiul Nobel pentru chimie (1997) pentru descoperirea funcţionării ATP sintetazei.
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Modelul rotaţional al lui Boyer
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Halobacterium halobium
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Experienţa lui Racker şi Stoeckenius
ATP-sintetaza ↔ Pompă De H+
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15.6. Transportul ionilor prin membrana mitocondrială internă
a) Translocarea ATP / ADP → ATP-ADP translocaza
b) Transportul anionilor: piruvat, fosfat anorganic
c) Transportul aspartat / glutamat
d) Transportul acizilor graşi → carnitină
e) Transportul Ca2+ → Ca2+-ATP-aza
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Oxaloacetate
Malate
α−Κetoglutarate
Glutamate
Aspartate
NADH
NAD+
Glutamate
Aspartate
Malate
NADHNAD+
Oxaloacetate
α−Κetoglutarate
CYTOSOL MATRIX
Transportul cuplat aspartat - glutamat
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15.7. Biogeneza şi originea evoluţionară a mitocondriei
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Genetic and transcription map of human mtDNA. The two internal circles represent both mtDNA strands with the encoded genes. External circles represent the RNAs transcribed from the heavy strand (filled lines) and light strand (open lines). 12S and 16S, ribosomal RNAs 12S and 16S; ND1 to ND6, subunits 1-6 of NADH dehydrogenase (complex I); cyt b, cytochrome b subunit of complex III; CO I, CO II and CO III, subunits of cytochrome coxidase (complex IV); ATPase 6 and ATPase 8, subunits of ATP synthetase (complex V). tRNA genes are indicated by the three letter code of the corresponding amino acid. H1, H2 and L indicate transcription initiation points for the H- and L-strand, respectively. OH and OL represent replication origins for the H- and L-strand, respectively. Arrows indicate the transcription or replication direction.
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Proteine mitocondriale sintetizate în mitocondrie
PorinaMembrana externă
Citocromul cCitocromul bSubunitatea 4 a CoQH2-citocrom c reductazei (citocromul c1)
Spaţiulintermembranar
Transportorul ADP-ATPTransportorul Fosfat-OHSubunităţile 4,5,6 şi 7 ale citocrom c oxidazeiSubunităţile 1,2,5,6,7,8 ale CoQH2-citocrom c reductazei
Membrana internă
F1-ATP-aza: Subunităţile a,b,d, gADN, ARN polimerazele mitocondrialeProteinele ribosomaleCitrat-sintetazaOrnitin-aminotransferazaAlcool-dehidrogenaza
Matrice
PROTEINALOCALIZARE
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Florea & Craciun, 2012
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ADNmtplasmide
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15.8. Implicaţiile mitocondriilor în patologie
A. Boli genetice mitocondriale-mutaţii în mtDNA, mutaţii în genele nucleare pentru proteinele mitocondriale
- mitocondrii mari, ineficiente energetic; ţesuturile cele mai sensibile: muşchiul scheletic, cardiac, SNC
→ encefalomiopatii mitocondriale (la pacienti tineri sau adulti)
→ citopatii mitocondriale: ficat, rinichi (în copilărie)
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Exemple de boli genetice mitocondriale:
-Boala Leber – atrofia nervului optic – orbire rapidă(la orice vârstă)
-Sindromul MERF (myoclonal epilepsy with ragged fibres) : epilepsie, aspect specific al fibrelor musculare
(în copilărie)
-Sindromul MELAS (mitochondrial encephalopathy with lactic acid and stroke episodes) - debut între 5-15 ani
-Boala Luft decuplare fosforilării oxidative - căldură
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MERF
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MERF
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B. Alte procese patologice în care sunt implicate mitocondriile
→ mitocondriile sunt implicate în toate bolile
→ ADN-ul mitocondrial nu este protejat - expus speciilor reactive ale oxigenului (radicali liberi implicaţi în patogeneza tuturor bolilor (cardiovasculare, cancer, diabet...)
→ mitocondriile – implicate în primii paşi ai apoptozei
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