mitocondria

What are the main functions of mitoxondria

1. Thermogenesis

2. Growth

3. Atp

4. Caclium homeostasi

5. Redox regulation and cell signaling

What can happen when mitocondria is dysfunctional

1. Ros

2. Inflammation

3. Cell death

In genetic mutation involving structural mutation what is involved

Cardiolipin

The inner membrane fold alre called?

The internal part is the…

Cristae

Matrix

How does the permbability of the inner and outer membrane chages

The inner to mantain protion gradient is highly IMPERMBERABLE to small molecule

The outer is VERY PERMBERABLE to small molecules and you have PROINS for free small molecule

Why is mitocondria associated with

Microtubules and in contact with er

Microtubulues allow the movemnet and organization of mitocondria

Er to exchange lipids

From where do mitocondria get

1. Iron

2. Calcium

3. Lipids

Fe from endosomes from endocytosis

Calcium from er

Lipid from er and lipid droplets

What mediates lipid tranfer between er and mitocondria filippase and scramblase?

No PHOPHOLYPID TRANSFER PROTEINS

That substance synthetized my mitocondira improves oxidative phorylation

Cardiolipin

THAT RESTRICTS THE PROTON FLOW ACROSS THE INNER MEMBRANE

Most of the proteins imported

Where are synthstized?

Is it co or post translational translocation??

What is required for their transport?

Are made in FREE ribosomes

They arrive as complete polypeptides ( POST translational translocation!!!!) and unfolded by hsp70!

Bip pulls them in using atp

Protein translocation requires eletrochemical potential accros the inner membrane

Some have pre sequence other internal import signals

Which kind of translocation is for protein directed here

POST TRANSLATIONAL TRANSLOCATION

BIP, HISTON 70 PULLS THEM IN

Eletrrochemical gradient acrros the inner membrane is needed

Most proteins are imported by TRANSLOCASE

Outer, inner are called?

Translocase of inner membrane is TIM (t inner)

Translocase outer (t OuterM) tom

You have a protein that has to get from the intermembrane space inside the matrix what do you use

Tim

From where does the energy for translocation comes from

1. From membrane potential ( for tim)

2. From atp hydrolisi( es, one for hsp70)

3. From redox potenstial ( fro tom)

Conditions for having a spontanous reaction

Entropia neg entalpia positiva e gibs negativa

Se hai A B e gibs e negativa sara a verso b se è poistiva il contrario B A

Atp hydrolysisi if vaourable or not

YES atp hydrolysis is favourable

Glycolisis in summary what is transported to mitocondria? Net of atp

Anaerobic

Cytosol

Piruvate 2 nadh and 4 atp ( ma ne usi due ) net è 2 atp

Of there is oxigen you bring piruvate into mitocondria

Oxidative phoph. Of pyruvate how does it enters the krebs cycle

Acetil coa+ co2+ 2 NADH

The citric acid cycle complets the oxidation of glucose to 6 molecules of co2 how much for each cycle

1 ATP, 3, NADH, 1, FADH2

HOW MUCH ATP IN OXIDATIVE PHOPHRYLATION IN TOTAL

34 ATP

Which are the 4 complexes of the elctron transport chain?

1. Nadh transferes electron to ELCTRON TO COMPLEX I

2. NADH dehydrognease IS COMPLEX I

3. Complex II recives through ubiquinon from fadh2

2. Cytocrome c reductase

3. Cytrocome c oxidase

4. FADH TRANSFERS ELECTRONS TO COMPLEX 2

Through ubiquinon

The passage of electrons thorugh the electron chain and the rtransport of protons out of the internal part of mitocondria generates

CHEMIOSOMOTIC COUPLING

What is complex V

Does it transport electrons or recives from. Nadh?

Complex v DOES NOT TRANPORT ELECTRONS

It uses the energy of the chemiosomtic coupling to create ATP from adp and p

COMPLEX v is atp synthease

Atp synthease ( complex V) is formed by 2 complexes f1, f, 0 charcateristics of these complexs?

F0 forms a channel where proton pass

F1, takis the energy to synthteixe atp

Total of atp?

2 from glycoslisis net by SUBSTRATE LEVEL PHOPHORYLATION

2 atp that depend on the elcgtron shuffle that transport nadh

2 atp from krebs also substrate level

34 oxid. Php.

Where are the elctron transport chain and the atp syntehease

INNER MITROCONDRIAL MEMBRANE

Pumping back protons in the matrix

What does the matrix contain

Dna

Enzymes for oxidative phophorylation. F pyruvate into CO A

In the matrix is where the krebs cycle is

The last step is in the matrix or the inner memebane

In the inner membrna eyou have electron chian

In the matrix you have the krebs cycle

Pyruvate import into the matrix from the inner membrane space is due to?

PH GRADIENT DRIVES PIRUVATE IMPORT

Atp and adp exchanges in the matrix are driven by

Voltage gradient

Mammalian mitochondrial genome how many protein does it encode? The vast majority of the protein where are encoded

Mitocondrial just encodes some protein 13 for oxidative phophrylation and electron transport FEW the majority is from nucleus

The 1500 other proteins that mitocondria need are encoded in the NUCLEAR GENOME

That encodes the majority of the proteins for eòectron chains, most of the proteins that are needed for mitocondrial replication and expression and all the enzymes for its metabolism!!!!

What does the nuclear genome encode for mitocondria

That encodes

the majority of the proteins for eòectron chains,

most of the proteins that are needed for mitocondrial replication and expression

and all the enzymes for its metabolism!!!!

How do the protein from the nucleus get to mitocondria

Tim , tom

The chaperons hsp 70 pulls it in

What is heteroplasmy

Is a mutation in mtDna that creates a cell with a mix of genome

A mutation in mitocondria will it alwys be ohenotypicallly expressed?

No just if it goes above a certain thresold

Other wise it can be lieve o assente

Clinically mitocondrial disorder vary a lot dìfrom tissue to tisseu why?

Depends on the number of mitate genome and on who much that cell relies on energy

Tissue with different percentage ( and effct so) of mutate mt genome can be generated

What is leber hereditary optic neuropathy

Rapid loss of vision

Mutaation that alyter the electron transport chain

Leigh syndorm

Come la figlia di vivian leigh

Often fatal infntil myopathy due to progressive degeneration of motror, vision

GENE AFF. For oxidative phophorylation

NARP

Neuropathy, ataxia, retinitis pigmentosa

Mutation in function of atpase subuinny

MERF

Myoclonic epilepsy asosciated with ragged red fibers

Gene encoding leucy-t-rna

Muscle fiber with abnormally shaped mitocondria with crystals

What kind of reaction allow to breakdown substrates in peroxisomes prtectingfrom h202

OXIDATIVE reaction

Is cholesterol only synthetized in the SER?

No! The EARLY stage so making all the intermedita eproducts necessary for the SER is done in peroxisomes

Is bile and cholesterol synthesis in all cell

Liver cells

Peroxisomes also makes PLASMOLOGENS whata re they

Phopholyipds with one carco chain with eter or ester bodn

What enzyme converts hydrogenperoxide into water or other

CATALASE

Peroxides differences with mitocondria

1. Membrane

2. Dna

3. Protein synthesis site

4. Replication

1. Membrane is single inperoxixde and double in mitocondri

2. Peroixed do not have their own genome

3. No endosymbiosi

4. Protein are those from ribosomes free or er ( not do their own or from nucleus)

5. Peroxides can replicate by division adn also REGENERATED IF LOST

In response to different metabolic needs

Which are the 2 way peroxisomes can be formed

1. Vescicle budding from er and with fusion

2. Growth of pre existing which is more rapid

How does the transport from proteins made in the cyto?

Signals and receptors?

Is it with pores or translocators?

Pts1 and pts2 signals

TRANSLOCATOR PEROXIN

Atp dependednt mechanis,