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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
how to treat refsum disease
diet | plasmapheresis
26
XR disorder of beta oxidation Progressive disease that can lead to adrenal gland crisis, coma, death
Adrenoleukodystrophy | mutated ABCD1 gene
27
What do you get a build up of in Adrenoleukodystrophy
VLCFA in adrenal glands, white matter, testes
28
ubiquitin-proteasome system have been implicated in some cases of
Parkinson disease
29
type of filament involved in muscle contraction and cytokinesis
microfilaments - actin - microvilli
30
type of filament involved in maintaining cell structure
intermediate filaments - vimentin - desmin - cytokeratin - lamins - GFAP - neurofilaments
31
type of filament involved in movement and cell division
microtubules - cilia - flagella - mitotic spindle - axonal trafficking - centrioles
32
Dynein
retrograde to microtubule ( + to -)
33
Kinesin
anterograde (- to +)
34
Drugs that act on microtubules
``` Mebendazole (anthelminthic) Griseofulvin (anti fungal) Colchicine Vincristine/Vinblastine Paclitaxel ```
35
what allows for coordinated ciliary movement
gap junctions
36
Kartagener's syndrome can cause what in a woman
ectopic pregnancy | infertility
37
Kartagener's syndrome can cause
``` bronchiectasis recurrent sinusitis chronic ear infection conductive hearing loss situs inversus ```
38
How to screen for Kartagener's (AR)
decreased nasal nitric oxide
39
Problems forming triple helix during glycosylation of pro-alpha-chain hydroxylysine in the cytosol causes
osteogenesis imperfecta
40
Vitamin C deficiency affects
hydroxylation of specific proline and lysine residues for collagen synthesis in cytosol
41
what two processes of collagen synthesis happen extracellularly
proteolytic processing | cross-linking
42
what is collagen proteolytic processing
cleavage of disulfide rich terminal regions or procollagen ---> insoluble tropocollagen
43
problems with proteolytic processing of collagen causes
Ehlers-Danlos
44
What is collagen cross-linking
reinforcement of many staggered tropocollagen molecules by covalent lysine-hydroxylysine cross-linking by copper-containing lysol oxidase to make collage fibrils
45
problems with collage cross-linking causes
Ehlers-Danlos | Menkes disease
46
MC genetic defect in osteogenesis imperfecta
COL1A1 | COL1A2
47
Classical type (joint and skin) of Ehlers-Danlos genetic mutation
type V collagen COL5A1 COL5A2
48
Vascular type Ehlers Danlos
type II pro collagen | COL3A1
49
Genetic defect in Menkes disease
defective Menkes protein | ATP7A
50
Brittle kinky hair growth retardation hypotonia
Menkes Disease
51
Menkes disease is caused by
impaired copper absorption and transport decreased activity of lysol oxidase defective collagen
52
MC type of Ehlers-Danlos
hyper mobility type
53
MC type of osteogenesis imperfects inherited usually
AD decrease production of otherwise normal type I collagen can have hearing loss (abnormal ossicles)
54
What is elastin
stretchy protein in | skin, lungs, large arteries, elastic ligaments, vocal cords, ligaments flava
55
elastin is rich in
NONhydroxylated proline, glycine, lysine
56
elastin is normally broken down by
elastase
57
elastase is inhibited by
alpha-1-antitrypsin
58
changes with aging
decreased dermal collagen and elastin decreased collagen fibrils cross linking stays the same
59
Subluxation of eyes in Marfans
upward and temporally (vs. down and in w/homocystinuria)
60
MCC of death in Marfans
dissection and HF
61
Marfan syndrome genetic mutation
AD FBN1 gene on chr 15 defective fibrillin
62
MC presentation of Marfan's
MVP and cystic degeneration of aorta | aortic aneurysm

UWorld Review (35 decks)

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