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19 Cards in this Set

  • Front
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Mitochondria Structure

double membrane system - inner membrane and outer membrane separated by inter membrane space


cristae - numerous folds of inner membrane


matrix - interior of mitochondria, containing mitochondrial genetic system as well as enzymes responsible for: 1) Krebs 2) Beta Oxidation of FA 3) Pyruvate --> Acetyl CoA


Inner membrane is principle site of oxidative phosphorylation and ATP generation

Genetic System of Mitochondria

circular genomes, multiple copies per organelle


encode tRNAs, rRNAs, and only a small number of proteins that are essential components of oxidative phosphorylation. Most mitochondrial proteins are encoded by nuclear genome


Use slightly different code than "universal" genetic code used by prokaryotic and eukaryotic cells

Cellular Respiration

C6H12O6 + O2 = CO2 + H2O + 36 ATP




(1) Glycolysis


(2) Krebs Cycle


(3) Oxidative Phosphorylation

Glycolysis

In cytoplasm glucose is converted to pyruvate


Pyruvate --> Acetyl CoA (in mitochondria)

Krebs Cycle


Citric Acid Cycle

in matrix, production of high energy electrons in NADH and FADH2


Two carbons are oxidized --> CO2, and energy from these reactions is stored in form of NADH and FADH2 and some GTP synthesis of reducing nucleotides

Oxidative Phosphorylation

electrons are transferred from NADH or FADH2 to molecular oxygen via electron transport chain proteins in inner mitochondrial membrane


As a result of this flow of e-, H+ pumped from matrix to intermembrane space


Generates a pH gradient --> potential energy


-Proteins flow back to matrix via ATP synthase via chemiosmotic coupling (e- transport --> ATP)


Glycolysis + Citric Acid Cycle + Oxidative Phosphorylation = 36 ATP

Porins

proteins that form channels to allow free diffusion of molecules smaller than 1kD into highly permeable outer mitochondrial membrane


import into mitochondria is posttranslational

Import into Mitochondria

pre sequences are amino-terminal sequences of 20-35 aa that target protein to mitochondria


Mitochondrial sequences are recognized by receptor on outer membrane and cleaved by matrix processing pepsidase (MPP) in matrix proteins


complexes in inner and outer membrane coordinate


TOM and TIM associate physically so protein can pass right through.


Proteins must be a least partially unfolded, so members of Hsp70 proteins help in partial unfolding and translocation


Additional mechanisms are in place besides MPP to direct proteins to correct submitochondrial compartment

Mitochondrial Diseases

affect function of mitochondria and/or due to mitochondrial DNA


Unique characteristics --> non mendelian, from mother. Critical to cell function.


Becomes apparent only once the number of affected mitochondria reaches a certain level


-Copies of DNA present are divided randomly, so if only a few copies are defective, may only end up in one mitochondria


-Lack of error-checking capability


Worst in muscles, cerebrum, and nerves --> need most energy

Diabetes Mellitus (DAD)


Maternal Inherited Diabetes and Deafness (MIDD)

mitochondrial disease associated with mitochondria defective gene

Leber's Hereditary Optic Neuropathy (LHON)


or


Leber Optic Atrophy

mitrochondrially inherited degeneration of retinal ganglion cells (RGCs) and their axons that lead to an acute or subacute loss of central vision

Chloroplast

Responsible for photosynthesis


double membrane called chloroplast envelope


thylakoid membrane forms network of flattened disks called thylakoids, arranged in stacks called grana. Inside thylakoid is thylakoid lumen\s


Stroma is area between envelope and thylakoid membrane


Gradient is against thylakoid membrane


Green color is due to chlorophyll


Have their own genetic code --> ~30 proteins in photosynthesis


3500/~95% of proteins still encoded by nuclear genes

Import of Proteins into Chloroplast Stroma

Transit peptides are N-terminal sequences of 30-100 aa that direct protein translocation across the two membranes of chloroplast membrane


A guidance complex initially recognizes the transit peptides and directs them to the translocase of chloroplast outer membrane


- TOC complex: translocate of outer membrane


- TIC : of inner membrane


- Stroma Processing Peptidase (SPP) cleaves transit peptides from proteins imported to the chloroplast stroma

Plastids

family of plant organelles that have DNA


chloroplasts


chromoplasts - lack chlorophyll but have carotenoids, responsible for yellow, orange, and red colors


leucoplasts - nonpigmented, store energy in nonphotosynthetic tubular internal membrane in dark - thylakoid


etioplasts - intermediate stage of chloroplast in which chlorophyll has not been synthesized


amyloplasts - store starch


elaioplasts - lipid storage


development is controlled by both environmental signals and intrinsic programs of cell differentiation

Photosynthesis

energy from sunlight harvested and used to drive glucose synthesis from CO2 and H2O


6CO2 + 12H2O + light --> C6H12O6 + 6O2+6H2O


Light reaction in thylakoid


Dark reaction in stroma


Affected by CO2 concentration, light intensity, and temp

Electron flow through Photosystems I and II

LIGHT REACTIONS


PSI&II are multi protein complexes in thylakoid membrane involved in light reactions


Starts at PSII. Chlorophyll absorbs light and loses a high energy e- that travels down chain to create gradient


Chlorophyll regains its e- by taking an O from H2O.


For every pair of e-, H+ transferred across thylakoid membrane at cytochrome bf complex. Oxidation of H2O --> protein gradient which drives ATP synthesis (when gradient reaches critical value - ATP synthase-->ATP)


Produces NADPH but upon cyclic flow can make ATP if needed

Cyclic Electron Flow

From PSI


Produces ATP without NADPH


Oxygen is only PII


NADPH --> PI


Used in dark reaction for sugars

Light Independent Reaction

dark reaction / Calvin Benson Cycle


Use high energy molecules produced in first phase to capture carbon dioxide (CO2) and make 3-carbon sugars which are later combined --> glucose


--> Dark Reaction is affected by light intensity because light reaction is "Carbon Dioxide Capture"


--> 1 molecule of glucose

Peroxisomes

small, single membrane enclosed organelles that contain enzyme involved in a variety of metabolic reactions


50 different enzymes: oxidative --> catalase (decompose H2O2)


2H2O2--> 2H2O + O2 eliminate poisonous hydrogen peroxide detox toxic substances in blood


Breakdown of Long FA Molecules --> beta oxidation


(in animals and mitochondria) (yeast & plant --> exclusive for peroxisome)


Proteins are selectively imported in.


Peroxisome Targeting Signals (PTS)


Peroxins - peroxisomal proteins (32 of them) participate in process of importing peroxisomal proteins in ATP and peroxisome assembly


--> lipid metabolism --> Zellweger Syndrome: brain.


Pex2 - one form of syndrome. Or milder --> lack of recognition signals