Cellular I

Question 1

M phase

Answer 1

mitosis and cytokinesis

Question 2

cyclin-CDK

Answer 2

CDK - inactive

cyclin - activate CDK

Question 3

regulate G1 > S

Answer 3

p53 and hypo-P Rb inhibit progression

Question 4

permanent cell

Answer 4

remain G0 - neuron, skeletal m, cardiac m

Question 5

stable cell

Answer 5

quiescent - enter G1 from G0

Question 6

labile cell

Answer 6

never enter G0 - rapid division - most affected by chemo

bone marrow, gut epithelium, skin, hair follicles, germ cells

Question 7

rough ER

Answer 7

synthesis secretory protein

Question 8

nissl bodies

Answer 8

RER in neurons - NT for secretion

Question 9

free ribosome

Answer 9

synthesis of organellar and cytosolic proteins

Question 10

smooth ER

Answer 10

steroid synthesis and detoxification

Question 11

golgi modification

Answer 11

N-oligosaccharide on aspargine
add O-oligosaccharide on serine and threonine
add mannose-6-P to protein for trafficking to lysosome

Question 12

mannose-6-phosphate

Answer 12

mark protein traffic to lysosome

Question 13

I cell disease

Answer 13

inclusion cell disease
-inherited lysosomal storage disease

defect N-acetylglucosaminyl-l-phosphotransferase

failure phosphorylate mannose residues - mannose 6-P
-protein secrete extracellular - instead of delivered to lysosome

Question 14

coarse facial features, cloudy cornea, restricted joint movement, high plasma lysosomal, fatal in children

Answer 14

inclusion cell disease

-defective phosphorylation manose residues - no trafficking to lysosome

Question 15

peroxisome

Answer 15

catabolism - very long chain fatty acids, branched chain fatty acids, amino acids

Question 16

proteasome

Answer 16

degrade damaged or ubiquitin tagged protein

Question 17

defect ubiquitin proteasome

Answer 17

seen in some cases parkinsons

Question 18

collagen synthesis pathway

Answer 18

RER synthesis - preprocollagen
> GLY - proline - lysine
> hydroxylation of proline and lysine (Vit C - scurvy)
> glycosylstion of hydroxylysine residues
> form procollagen - via H and disulfide bonds - triple helix (osteogenesis imperfecta - impaired triple helix)
> exocytosis

outside cell - proteolytic processing
> cleavage procollagen - makes insoluble
> reinforce tropocollagen - cross link lysine-hydroxylysine - enzyme lysyl oxidase (copper containing)

Question 19

glycine content

Answer 19

1/3 collagen

Question 20

ehler danlos syndrome

Answer 20

problem crosslinking collagen

defective lysyl oxidase

Question 21

lysyl oxidase

Answer 21

cross link collagen in ECM

-defective in ehler danlos syndrome

Question 22

vit C deficient

Answer 22

scurvy - cannot hydroxylate proline and lysine of collagne

in the RER of cell

Question 23

osteogenesis imperfecta

Answer 23

auto dom
-decreased production type I collagen (normal collagen)

blue sclera
multi fractures
hearing loss - abnormal ossicles
dental imperfection

Question 24

types of ehlers danlos

Answer 24

type 1 - MC - hypermobility
type 2 - classic - joint and skin - collagen type V
type 3 - vascular - type III collagen

Question 25

menkes disease

Answer 25

X-linked rec - impaired copper absorption defective menkes protein - ATP7A decreased activity lysyl oxidase (copper is cofactor) brittle - kinky hair, growthy retardation, hypotonia

Question 26

elastin

Answer 26

rich nonhydroxylated proline, glycine, lysine tropoelastin with fibrillin scaffold cross linking - occurs extracellularly elastase - breaks it down - inhibited by a1 antitrypsin

Question 27

marfan

Answer 27

defect fibrillin | -glycoprotein sheath around elastin

Question 28

emphysema

Answer 28

a1 antitrypsin deficiecny | -excess elastase activation

Question 29

wrinkles of aging

Answer 29

decreased collagen and elastin production

Question 30

actin

Answer 30

microfilament

Question 31

intermediate filaments

Answer 31

maintain cell stucture vimentin, desmin, cytokeratin, lamins, GFAP, neurofilaments

Question 32

microtubules

Answer 32

movement and cell division mitotic spindle axon transport centrioles

Question 33

desmin

Answer 33

intermediate filament of muscle

Question 34

vimetin

Answer 34

intermediate filament of CT

Question 35

cytokeratin

Answer 35

intermediate filament of epithelial cells

Question 36

GFAP

Answer 36

intermediate filament of neuroglia

Question 37

neurofilaments

Answer 37

intermediate filament of neurons

Question 38

microtubule structure

Answer 38

heterodimer alpha and beta tubulin dimer - 2 GTP bound

Question 39

dynein

Answer 39

retrograde = + to - on microtubule

Question 40

kinesin

Answer 40

anterograde = - to + on microtubule

Question 41

drugs act on microtubule

Answer 41

``` colchicine paclitaxel vincristine/vinblastine mebendazole griseofulvin ```

Question 42

cilia structure

Answer 42

9 + 2 arrangement axonemal dynein - ATPase that linkes peripheral 9 doublets and causes bending of cilium

Question 43

kartagener syndrome

Answer 43

primary ciliary dyskinesia defect dynein arm infertility - immotile sperm and dysfunctional fallopian tube cilia increased risk ectopic situs iversus, bronchiectasis, recurrent sinusitis

Question 44

fungal membrane

Answer 44

ergosterol

Question 45

Na/K pump

Answer 45

3 Na out of cell 2K into cell requires ATP

Question 46

digoxin MOA

Answer 46

inhibit Na/K ATPase - leads to indirect inhibition of Na/Ca exchange - increased Ca increased cardiac contractility

Question 47

ouabain

Answer 47

inhibit Na/K ATPase binds K binding site

Question 48

most abundant protein human body

Answer 48

collagen

Question 49

type I collagen

Answer 49

bone, skin, tendon late wound repair - replace type III

Question 50

type II collagen

Answer 50

cartilage, vitreous body, nucleus pulposus

Question 51

type III collagen

Answer 51

reticulin, skin, blood vessel, uterus, fetal tissue, granulation tissue (early wound - replaced by type I)

Question 52

type IV collagen

Answer 52

basement membrane

Question 53

alport syndrome

Answer 53

defective collagen type IV

Question 54

goodpasture syndrome

Answer 54

target collagen type IV BM

Question 55

vascular ehler danlos

Answer 55

type 3 collagen type III defect - vascular