Peroxisome, Mitochondrion, Lysosome

Question 1

Peroxisome

Answer 1

break down of long chain fatty acids and ether synthesis

Question 2

Long chain fatty acid break down

Answer 2

via beta oxidation

releases H2O2 which is broken down via peroxidase

Question 3

ether synthesis

Answer 3

makes plasmologen which is used by oligodendrocytes to make myelin (brain. leads to neurologic defects)
backbone for lipid membrane

Question 4

DAB

Answer 4

diaminobenzadine
stains peroxisome by being taken in via enzyme with H2O2
leads to brown precipitate and is very electron dense so shows up as dark spot in peroxisome

Question 5

regulation of peroxisomes

Answer 5

increase and decrease according to the needs of the cell
when more peroxisome is needed, genes are transcribed and protein is made. protein then fuses with existing peroxisome and that peroxisome multiplies
number of peroxisomes and size can be controlled in cell

Question 6

PTS1

Answer 6

peroxisome targeting signal #1

serine-lysine-leucine sequence on C’ terminus of any protein will tag that protein for transport to peroxisome

Question 7

PPARs

Answer 7

peroxisome proliferator activated receptor
FFA will bind to FABP and enter the nucleus which will then bind to PPAR
RA will bind to RXR (retinoid acid receptor) and interact with PPAR which will then sit on PPRE (peroxisome proliferator response element) and alter transcription of genes downstream
Exercise will upregulate PPARS

Question 8

PDK4

Answer 8

Pyruvate dehydrogenase lipoamide kinase isozyme 4
through exercise, PPARs will bind to FFA and increase uptake of FFA into the cell
Also will turn on production of PDK4 which will inhibit utilization of glucose and burn FFA as source of energy in mitochondria

Question 9

CD36

Answer 9

allows FFA to enter into cell (relevant when exercising and muscle cells want to burn lipids instead of glucose through PPAR pathway)

Question 10

PEX

Answer 10

proteins responsible for importing enzymes into peroxisome
will bind to any protein that has PTS (serine-lysine-leucine at C’ terminus) and bind to membrane of peroxisome and go inside
x-linked
transporter of long chain fatty acids

Question 11

PTS1

Answer 11

bind to PEX5

Question 12

PTS2

Answer 12

bind to PEX7

signal at N terminus of protein but signal is not as conserved (not as specific)

Question 13

x- linked adrenoleukodystrophy

Answer 13

increase in very long chain FFA in blood

restrict diet and use lorenzo’s oil

Question 14

mitochondria

Answer 14

powerhouse!

Question 15

intermembrane space

Answer 15

in mitochondria between inner and outer membrane

ionically and pH wise same as cytosol so as to allow ions to flow freely in and out of membrane

Question 16

inner matrix

Answer 16

space within inner membrane

location of mitochondrial DNA and have ribosomes to make mitochondrial proteins

Question 17

cristae

Answer 17

invaginations in mitochondria

Question 18

inner membrane

Answer 18

impermeable die to specialized membrane lipids
not even proton can pass
causes pH of inner matrix to be alkaline

Question 19

How is ATP formed in mitochondria

Answer 19

through H+ gradient
H+ are pushed out through the electron transport chain (using energy in electron) into intermembrane space (creates electrochemical gradient between matrix and inner membrane space) where the H+ will go back down through the ATP synthase to turn ADP into ATP

Question 20

acidification of endosome and lysosome

Answer 20

ATP synthase rotor moves in reverse and burns ATP to move H+ out of matrix

Question 21

mitochondrial size

Answer 21

changes via fusion or fission

Question 22

lysosomes

Answer 22

acidic vesicles in the cytoplasm full of hydrolytic enzymes

pH 4.5-5

Question 23

function of lysosome

Answer 23

hydrolyze all macromolecules to momeric subunits and recycle subunits via transmembrane transport to cytoplasm

Question 24

hydrolase

Answer 24

require acidic environment and are inactive outside lyososome

Question 25

how are vesicles brought into lysosome?

Answer 25

through endosome

Question 26

tagging protein for transport to lysozyme

Answer 26

tagged by M6P (mannose 6 phosphate) will bind to receptor in trans Golgi which is then packaged into vesicle and directed to early endosome which is becoming acidic via ATP pump
the lower pH- enzyme will let go of receptor and receptor will be shipped back to golgi
over)

Question 27

lysosomal storage disease

Answer 27

problems in protein transport will lead to disease which will result most often neurologically- the material needing to degrade will build up because of no turn;
either due to lack of lysosomal enzymes or lack of enzymes needed to tag

Question 28

I cell disease

Answer 28

inclusion cell disease, also known as mucolipidosis II
defective glcnac phosphotransferase which is used to tag enzymes to enter into lysosome
death by age 7; developmental delays; hepatomegaly, cognitive delay

Question 29

hepatomegaly

Answer 29

enlarged liver; in I cell disease it is because stuff is not getting degraded

Question 30

autophagosome

Answer 30

mitochondrial death