CB 5

Question 1

MERRF- Myoclonic epilepsy with ragged red fiber

Answer 1

• red appearing mitochondria
• Gomori trichrome stain
• ragged red fiber appearance to muscle fiber
• parking lit inclusion in mitochondrion
• symptoms: myoclonus, seizures, ataxia

Question 2

functions of perioxisomes

Answer 2

• b oxidation of free fatty acids
• plasmalogen synthesis
• regulate H2O2
• kill pathogens
• oxidize EtOH

Question 3

Zellweger syndrome

Answer 3

impaired import of enzymes into peroxisomes, defective import protein on the unit membrane of the peoxisome

Question 4

cytoskeletal elements

Answer 4

• microtubules

- filaments

Question 5

Filaments

Answer 5

• actin= thin
• intermediate
• myosin= thick

Question 6

kinesine

Answer 6

microtubule motor

Question 7

botulinum toxin

Answer 7

works by proteolysis of SNARE proteins

Question 8

coated vesicles

Answer 8

clathrin
COPII
COPI

Question 9

COPII

Answer 9

anterograde transport from ER to cis golgi network

Question 10

COPI

Answer 10

retrograde transport from cis golgi network aka CGN

Question 11

retrograde transport is needed for….

Answer 11

returning membrane v SNARE proteins and missorted ER proteins back to the ER

Question 12

Vesicle snare [vsnare]

Answer 12

recognize target SNAREs

Question 13

SNARE proteins

Answer 13

allow vesicle to recognize its target domain

Question 14

Tetanus and Botulism

Answer 14

enter nerve terminals and proteolyze SNARE proteins, prevents vesicles containing neurotransmitters to release them

Question 15

Mitochondria

Answer 15

• acidophillic organelle
• located in areas of high metabolic activity
• erythrocytes don’t have any mitochondria

Question 16

Composition of mitochondria

Answer 16

• mainly protein
• lipid
• small amts of RNA and mtDNA
• *most mitochondrial proteins are synthesized by free polyribosomes in the cytoplasm and are actively transported in by translocator proteins

Question 17

Outer membrane of mitochondria

Answer 17

• fairly permeable, large channel forming proteins

- translocator protein

Question 18

Inner membrane of mitochondria

Answer 18

• selectively permeable
• decreased permeability due to presence of high conc of cardiolipin
• contains enzymes for ETS
• contains translocator proteins

Question 19

CO and Cyanide

Answer 19

block the ETS

Question 20

Intermembrane space of mitochondria

Answer 20

between the inner and outer mitochondrial membranes

Question 21

shelf like cristae

Answer 21

located in mitochondria

seen in hepatocytes and cardiac/skeletal muscle

Question 22

tubular cristae

Answer 22

found in:

steroid secreting cells

Question 23

intracristal space

Answer 23

is continuous with the intermembrane space

Question 24

Mitochondrial Maxtrix composition

Answer 24

enzymes for Krebs cycle
enzymes for fatty acid b oxidation
matrix granules

Question 25

Cytochrome c

Answer 25

release of this activates the apoptotic mechanism

Question 26

mutations in mitochondrial DNA

Answer 26

-diabetes, deafness, heart disease, alzheimers, parkinsons, LHON

Question 27

Peroxisomes

Answer 27

- contain oxidative enzymes for fatty acids - catalase: forms water and oxygen from hydrogen peroxide - oxidation of ingested ethanol - synthesis of plasmalogens!

Question 28

lipitor

Answer 28

leads to increased uptake of triglycerides and cholesterol, causes an increase in peroxisomes

Question 29

plasmalogen defects

Answer 29

lead to abnormalities of myelination in nerve cells

Question 30

Drugs that inhibit polymerization of microtubules

Answer 30

colchine, vinblastine, vincristine

Question 31

Taxol

Answer 31

inhibits depolymerization of microtubules

Question 32

Functions of microtubules:

Answer 32

- support to the cytoplasm for cell shape - organelle support - cell division - motility of cilia and flagella - long range transport of vesicles by microtuble motor proteins

Question 33

Microtubule motors

Answer 33

- kinesin | - dyein

Question 34

Kinesin

Answer 34

- motor towards the periphery - defect causes decrease transport of serotonin receptors - may cause anxiety disorders

Question 35

Dynein

Answer 35

motor that goes towards the nucleus from positive to negative end