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UWorld Review > cellular biochem > Flashcards

cellular biochem Flashcards

1
Q

What does p53 do

A

induces p21 –> inhibits CDKs

  • hypophosphorylation (activation of Rb)
  • inhibition of G1-S progression
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2
Q

Li-Fraumeni syndrome

A

mutation in p53 tumor suppressor gene = unrestrained cell division

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3
Q

Growth factors
insulin, PDGF, EPO, EGF
all bind

A

tyrosine kinase receptors to transition cell from G1 to S phase

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4
Q

Permanent cells

A 
neurons
skeletal and cardiac muscle
RBCs
regenerate from stem cells
remain in G0 phase
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5
Q

Stable (quiescent) cells

A 
hepatocytes
lymphocytes
PCT
periosteal cells
enter G1 from G0 when stimulated
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6
Q

Labile cells

A 
Bone marrow
gut epithelium
skin
hair follicles
germ cells
never go into G0, divide rapidly w/short G1
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7
Q

Cells most affected in chemo

A

labile cells

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8
Q

RER in neurons are called

A

Nissl bodies

make peptide Its for secretion

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9
Q

2 cells rich in rough ER

A
  • Mucus secreting goblet cells of small intestine

- antibody secreting plasma cells

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10
Q

site of steroid synthesis and detoxification of drugs/poisons

A

smooth ER

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11
Q

2 cells rich in smooth ER

A
  • liver hepatocytes

- steroid hormone producing cells of adrenal cortex/gonads

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12
Q

Cell trafficking/distribution center for proteins and lipids from ER to vesicles and plasma membrane

A

golgi

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13
Q

The golgi modifies N-oligosaccharides on

A

asparagine

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14
Q

The golgi adds O-oligosaccharides on

A

serine and threonine

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15
Q

The golgi adds mannose-6-phosphate to proteins for trafficking to

A

lysosomes

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16
Q

sorting centers for material from outside cell or from golgi

A

Endosomes

sends to lysosomes for destruction or back to membrane/golgi for further use

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17
Q

inherited lysosomal storage disorder

defect in N-acetylglucoasaminyl-1-phosphotransferase

A

I-cell disease (inclusion cell disease/mucolipidosis type II)

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18
Q

Presentation of I-cell disease

A
  • failure of golgi to phosphorylate mannose residues on glycoproteins
  • proteins secreted extracellularly rather than delivered to lysosomes
  • coarse facial features
  • gingival hyperplasia
  • clouded corneas
  • restricted movements
  • claw hand deformities
  • kyphoscoliosis
  • high plasma lysosomal enzymes
  • fatal in childhood
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19
Q

accumulation of protein in cytosol

A

absent/dysfunctional signal recognition particle

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20
Q

what is a signal recognition particle

A

abundant in cytosol

ribonucleoprotein

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21
Q

what does SRP do

A

traffics polypeptide-ribosome complex from cytosol to RER

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22
Q

beta-ox
alpha-ox
catabolism of branched chain FA, AA, ethanol
makes cholesterol, bile acids, plasmalogens

A

peroxisomes

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23
Q 
AR
disorder of peroxisome biogenesis
hypotonia
seizures
hepatomegaly
early death
A

Zellweger syndrome

mutated PEX genes

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24
Q 
AR
disorder of alpha oxidation
scaly skin
ataxia
cataracts/night blindness
shortening of 4th toe
epiphyseal dysplasia
A

Refsum disease phytanic acid not metabolized to pristanic acid

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25
Q

how to treat refsum disease

A

diet

plasmapheresis

26
Q

XR
disorder of beta oxidation
Progressive disease that can lead to adrenal gland crisis, coma, death

A

Adrenoleukodystrophy

mutated ABCD1 gene

27
Q

What do you get a build up of in Adrenoleukodystrophy

A

VLCFA in adrenal glands, white matter, testes

28
Q

ubiquitin-proteasome system have been implicated in some cases of

A

Parkinson disease

29
Q

type of filament involved in muscle contraction and cytokinesis

A

microfilaments

  • actin
  • microvilli
30
Q

type of filament involved in maintaining cell structure

A

intermediate filaments

  • vimentin
  • desmin
  • cytokeratin
  • lamins
  • GFAP
  • neurofilaments
31
Q

type of filament involved in movement and cell division

A

microtubules

  • cilia
  • flagella
  • mitotic spindle
  • axonal trafficking
  • centrioles
32
Q

Dynein

A

retrograde to microtubule ( + to -)

33
Q

Kinesin

A

anterograde (- to +)

34
Q

Drugs that act on microtubules

A 
Mebendazole (anthelminthic)
Griseofulvin (anti fungal)
Colchicine 
Vincristine/Vinblastine
Paclitaxel
35
Q

what allows for coordinated ciliary movement

A

gap junctions

36
Q

Kartagener’s syndrome can cause what in a woman

A

ectopic pregnancy

infertility

37
Q

Kartagener’s syndrome can cause

A 
bronchiectasis
recurrent sinusitis
chronic ear infection
conductive hearing loss
situs inversus
38
Q

How to screen for Kartagener’s (AR)

A

decreased nasal nitric oxide

39
Q

Problems forming triple helix during glycosylation of pro-alpha-chain hydroxylysine in the cytosol causes

A

osteogenesis imperfecta

40
Q

Vitamin C deficiency affects

A

hydroxylation of specific proline and lysine residues for collagen synthesis in cytosol

41
Q

what two processes of collagen synthesis happen extracellularly

A

proteolytic processing

cross-linking

42
Q

what is collagen proteolytic processing

A

cleavage of disulfide rich terminal regions or procollagen —> insoluble tropocollagen

43
Q

problems with proteolytic processing of collagen causes

A

Ehlers-Danlos

44
Q

What is collagen cross-linking

A

reinforcement of many staggered tropocollagen molecules by covalent lysine-hydroxylysine cross-linking by copper-containing lysol oxidase to make collage fibrils

45
Q

problems with collage cross-linking causes

A

Ehlers-Danlos

Menkes disease

46
Q

MC genetic defect in osteogenesis imperfecta

A

COL1A1

COL1A2

47
Q

Classical type (joint and skin) of Ehlers-Danlos genetic mutation

A

type V collagen
COL5A1
COL5A2

48
Q

Vascular type Ehlers Danlos

A

type II pro collagen

COL3A1

49
Q

Genetic defect in Menkes disease

A

defective Menkes protein

ATP7A

50
Q

Brittle kinky hair
growth retardation
hypotonia

A

Menkes Disease

51
Q

Menkes disease is caused by

A

impaired copper absorption and transport
decreased activity of lysol oxidase
defective collagen

52
Q

MC type of Ehlers-Danlos

A

hyper mobility type

53
Q

MC type of osteogenesis imperfects inherited usually

A

AD
decrease production of otherwise normal type I collagen
can have hearing loss (abnormal ossicles)

54
Q

What is elastin

A

stretchy protein in

skin, lungs, large arteries, elastic ligaments, vocal cords, ligaments flava

55
Q

elastin is rich in

A

NONhydroxylated proline, glycine, lysine

56
Q

elastin is normally broken down by

A

elastase

57
Q

elastase is inhibited by

A

alpha-1-antitrypsin

58
Q

changes with aging

A

decreased dermal collagen and elastin
decreased collagen fibrils
cross linking stays the same

59
Q

Subluxation of eyes in Marfans

A

upward and temporally (vs. down and in w/homocystinuria)

60
Q

MCC of death in Marfans

A

dissection and HF

61
Q

Marfan syndrome genetic mutation

A

AD
FBN1 gene on chr 15
defective fibrillin

62
Q

MC presentation of Marfan’s

A

MVP and cystic degeneration of aorta

aortic aneurysm

UWorld Review (35 decks)

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