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MCB I > Organelles > Flashcards

Organelles Flashcards

1
Q

Glycocalyx (Glycolipids & Glycoproteins) & CARBS can be found in

A

OUTER leaflet ONLY

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2
Q

What absorbs stain?

A

THICK CELL WALL on G+ PROK = purple

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3
Q

Which has cell wall and cell membrane? Prok or Euk

A

Prok

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4
Q

components of cell mem

A

lipids (phospholipids, glycolipids, cholesterol) &

proteins (transmembrane/integral and peripheral)

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5
Q

fn of mem proteins

A

RECEPTORS, tx, channels, & communication

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6
Q

phosphatidylcholine = __ (tails)

A

1 sat & 1 UNSAT tail (kink)

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7
Q

factors that INCREASE FLUIDITY

A

1) temperature
2) shorter chain
3) # on UNSAT fa tails

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8
Q

FA’s form what shape when put together

A

MICELLES

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9
Q

Phospholipids form what shape & whats holds together leaflets

A

BILAYER (VAN DER WAALS hold together leaflets)

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10
Q

what is the opposite of fluidity? Fluidity = easier to break

A

STABILITY

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11
Q

FLUIDITY imp 4

A

exocytosis
endocytosis
trafficking

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12
Q

phosphatidyl-serine can be found on outer or inner leaflet?

A

INNER

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13
Q

What INCREASES STABILITY of mem

A

CHOLESTEROL

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14
Q

FN of CHOLESTEROL on mem

A

INCREASES STABILITY

FILLS IN gaps when lipid = TOO FLUID

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15
Q

why do ACANTHOCYTES occur?

aka spur cells

A

= RBCs with TOO MUCH CHOLESTEROL

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16
Q

SPUR CELL ANEMIA / ACANTHOCYTOSIS

A

chronic alcoholics
hyperlipidemia
^^ reticulocytes
SYMP: portal HTN (caput medusae, jaundice) & asterixis (liver flap)

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17
Q

what is elevated in LIPID RAFTS

A

CHOLESTEROL & GLYCOPHINGOLIPIDS (less fluid)

stick out of mem

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18
Q

Glycosylphosphatidylinositol (GPI )is an example of ___

A

LIPID RAFT / glycolipid attaches 2 PM

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19
Q

what does FLIPPASE do and what energy source does it use?

A

flips phospholipids and uses NRG from HYDROLYSIS

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20
Q

in APOPTOSIS, what flips to make this occur?

A

PHOSPHATIDYL SERINE flipped 2 OUTER leaflet

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21
Q

GLYCOLIPID fn

A

cell 2 cell recog
protection
NERVE CONDUCTION
on OUTER leaflet only

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22
Q

lipid anchor / Peripheral protein fn

A

SIGNALING and ADHESION

can be on either side

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23
Q

What does GPI do?

A

LINK protein 2 outer leaflet

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24
Q

What are 2 methods of linking proteins to the INNER leaflet?

A

Fatty Acetylation and Prenylation

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25
Q

what reinforces the structure of RBCs?

A

SPECTRIN

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26
Q

what does SPECTRIN bind 2 on the inside of RBC?

A

Protein 4.1 and F-Actin

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27
Q

what binds band 3 to spectrin?

A

ANKYRIN

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28
Q

role of PRTEIN 4.1

A

link ACTIN - GLYCOPHORIN

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29
Q

HEREDITARY SPHEROCYTOSIS

A

SPECTRIN/ ANKYRIN/ P4.1 MUTATION
= loss of biconcave mem on RBC
symp= hemolytics anemia, splenomegaly, jaundine, gall stones
will lose parts of mem in every diapedesis

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30
Q

fn of CARBS on MEM

A

primary MARKER 4 cell recog

ex: L-selectin recog’s ADDRESIN

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31
Q

nucleus FN

A

compartmentalize.

PREVENT PREMATURE TL of pre-mRNA

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32
Q

what occurs at the nucleolus

A

rRNA synth and ribosome assembly

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33
Q

what do NPCs import?

A

snoRNP, snRNPs, & proteins (histones, polymerases & TFs)

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34
Q

what do NPCs export?

A

mRNA, tRNA & ribosomes

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35
Q

where is GAP located? GEF?

A

GAP in CYTOSOL

GEF in NUC

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36
Q

what is needed to import cargo?

A
  • NLS = nuclear localization signal
  • IMPORTIN
    GTP to UNBIND CARGO after imported to nuc
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37
Q

steps of importing cargo

A 
RAN-GDP binds 2 IMPORTIN
importin picks up cargo with NLS
move to nuc thru NPC
RAN-GEF bind 2 RAN-GTP
cargo UNBINDS
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38
Q

steps of exporting cargo

A

RAN-GTP binds 2 EXPORTIN
CARGO with NES binds to exportin
exportin+cargo moves to cyto
RAN-GAP causes GTP to HYDROLYZE = detatch cargo

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39
Q

what role does RAN-GAP have in exporting cargo?

A

GAP HYDROLYZES the gtp so the cargo can DETATCH

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40
Q

what does GTP do to importin and exportin

A 
Importin = allows cargo to detatch
Exportin = needed to PICK UP cargo
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41
Q

which lamin has its own gene?

A

Lamin B

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42
Q

what do lamins A&C form?

A

heterodimers

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43
Q

what is the role of EMERIN?

A

emerin binds to lamin which attaches to chromatin

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44
Q

what process degrades the PM 4 mitosis? then what happens to lamins?

A

PHOSPHORYLATION of Lamins!
Lamins A and C released as FREE DIMERS
Lamin B ANCHORED 2 inner mem!

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45
Q

Emery- dreyfus muscular dystrophy

A

rigid ELBOW, ANKLES and NECK

mutation in EMERIN or LAMIN A/C

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46
Q

dilated cardiomyopathy

A

LAMIN A/C defect = fragile nuc lamina

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47
Q

lipodystrophy

A

LAMIN A/C defect

^^ adipose in FACE & NECK. BODY= lean

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48
Q

what does Lamin A bind to?

A

architectural partners
chromatin
gene-regulating partners
signalling partner

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49
Q

HUTINCHIN GILFORD PROGERIA

A

ALTERED LAMIN A = unstable ncuclear envelope
= FORMS BLEBS & NPC CLUSTER
premature death of cells, accelerated againg
allopecia, prominent eyes, arteriosclerossi

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50
Q

snoRNPs

A

NUCLEOLUS

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51
Q

snRNPs

A

SPECKLES

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52
Q 
These symptoms are caused by what?
Kyphoscoliosis
Pectus carinatum
Normal iq
Cardiomegaly
A

Defective DEGENERATION OF KERATAN SULFATE

= MORQUIO SYNDROME

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53
Q

What are these symptoms caused by?
Recurrent infections
Hypopigmentation
Mild coag defects

A

DELAYED FUSION of phagosome with lysosome in leukocytes
-or-
DEFECT IN MICROTUBULE POLYMERISATION

MUTATION CHS1/LYST

This is Chediak-Higashi Syn

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54
Q

What cuases these syndromes and what is the name of the condition?

Progressive leukodystrophy in CNS & PNS
Paralysis
Cognitive decline
Progressive loss of muscle tone

A

↓ ARSA
ACCUMULATION OF SULFATIDES b’c can’t degrade

DESTROYS MYELIN

= Metachromatic Leukodystrophy

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55
Q

What causes these symptoms? And what is the name of the condition?
Aggressive behavior
Hyperactivity

A

Defect in HEPARAN SULFATE DEGRADATION

= sanfilippo syndrome

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56
Q

Differences between mucopolysaccharidoses diz’s: hunter and hurler

  • deficiency in what?
  • symptoms
  • inheritance
A

Hunter = need to hunt and aim for the x. So x- linked. And NO CORNEAL CLOUDING. Later onset and live longer
↓ in IDURONODATE SULPHATASE\

HURLER = ↓ alpha- L-IDURONODASE
CORNEAL CLOUDING. Retard. Hepatosplenomegaly, coarse face, hirsutism (abnormal hair growth)

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57
Q

What is the most severe form of mucopolysaccharidoses?

A

HURLER

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58
Q

What causes the following syndromes? Name of condition?
Psychomotor retardatioon
Big liver, spleen & heart valves
Chf or resp tract infection

A

↓ N-acetylglucosamine phosphotransferase → = no M6P tag

COMPLETELY EMPTY LYSOSOMES → accumulation of inclusion bodies

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59
Q

What Is the E that puts on the M6P tag? And where does it put the tag on?

A

e = N- acetylglucosamine phosphotransferase / GlcNAc

Does this at the cis-golgi

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60
Q

What does the V-type ATPase do?

A

Pumps H+ into lysosome to MAINTAIN ACIDITY

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61
Q

What protects the lysosome from digestion?

A

Lysosome is HEAVILY GLYCOSYLATED which prevents it from being digested

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62
Q

What does a MONOUBIQUITIN TAG do?

A

Makes protein go to MULTIVESICULAR BODY

Does this to DOWNREGULATE RECEPTORS

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63
Q

What is the purpose of a monoubiquitin tag?

A

To DOWN REGULATE RECEPTORS

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64
Q

what would you find in the DENSE FIBRILLAR CENTER of the nucleolus?

A

rRNA that is being TC’d -> cleaved and MODIFIED by snoRNPs

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65
Q

what would you find in the FIBRILLAR CENTER of the nucleolus?

A

transcriptionally INACTIVE Dna & pre-rRNA GENES

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66
Q

steps involved in co-TL translocation

A

1) protein needs ER sig sequence
2) SRP (signal recog particle) binds to the ERss and takes the ribosome with protien to the SRP-R on the translocon
3) protein goes into ER and SRP dissociates

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67
Q

what does BiP do?

A

= CHAPERONE

binds to protein and PULLS IT INTO ER

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68
Q

what is unique about using a INTERNAL sig seq?

A

1) can bing to translocon in 2 orientations

2) sig seq NOT CLEAVED

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69
Q

list rER resident proteins

A

DISULFIDE ISOMERASE (makes s-s bond on cysteins)
BiP
Calnexin
Calreticulin
last 3 = chaperones…last chance to fold correctly before DEGRADATION

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70
Q

where does the protein get polyubiquinated 4 degradation

A

in rER

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71
Q

what is N-LINKED GLYCOSYLATION

and what is the purpose?

A

adding a premade oligosacc to a protein so it can fold properly. protein sits on DOLICHOL (mem-bound lipid) until the protein thru translocon has an ASPARAGINE

THEN oligosaccharide tx to protein

THEN processed and LAST 4 sugars TRIMMED (3 glucose and 1 mannose)

PURPOSE = imp 4 correct FOLDING, TX & FN

72
Q

what is the signal that is needed for n-LINKED GLYCOSYLATION?

A

ASAPARAGINE in the protein

73
Q

Pt presents with rales, rhonchi, clubbed fingers and has had pneumonia multiple times, longer lung field
what is the mutation in? and what happens in this disease?

A

mutation in CFTR … deletion in F508 -> IMPROPER FOLDING

74
Q

what is the deficit in familial HYPERcholesterolemia

A

DEFECTIVE tx of LDL-R form ER –> Golgi.
so not sent up to the PM
it will not be able to RECOG LDL and bring it into lysosome to break it down to free cholesterol –> HIGH LDL if can’t break it down.

75
Q

what is the function of COP protein?

A

to DEFORM THE MEMBRANE so it can do FUSION

76
Q

COPI fn

A

take vesicle from golgi –> ER

77
Q

COPII fn

A

coat vesicles going from ER -> golgi

78
Q

Target signal KDEL, takes protein from nucleus to _____

A

ER

79
Q

Target signal KKXX, takes protein from nucleus to _____

A

ER

80
Q

Target signal M6P, takes protein from golgi to _____

A

LYSOSOME

81
Q

without an ER SIG SEQ, the protein will go where by default?

A

CYTOSOL

82
Q

Target signal NLS, takes protein to _____

A

NUCLEUS

83
Q

Target signal SKL, takes protein to _____

A

PEROXISOME

84
Q

in order for vesicle to FUSE with membrane, they need what signals??? what else is needed with the signals 2 complete fusion

A

SAR or ARF (G-proteins)

& they need GTP

85
Q

what is needed for GDP to turn into GTP for SAR/ARF to use them?

A

GEF & GTP causes G-protein to flip into membrane

86
Q

lists steps in VESICLE DOCKING

A

1) RAB-GTP allows 4 initial rxn in docking
2) RAB intereacts with Rab BINDING P -> docks & brings vesicle closer
3) V SNARE & T SNARE bind
4) both mem’s pulled close 2 each other
5) NSF/SNAPs untangle snares & v-snare gets recycled

87
Q

what puts on the m6p tag?

A

n-acetyl glucosamine phophotransferase

88
Q

a lack of GlcNAc (aka n-acetyl glucosamine phophotransferase) leads to what?

A

i-cell diz
= COMPLETELY EMPTY LYSOSOMES
accumulation of waste products
=coarse facial features, mental and growth retardation

89
Q

what is the main difference btw a vesicle that is new and one that is CLOSE TO PM?

A

CLOSE TO PM/OLDER = MORE ACIDIC & SMALLER

90
Q

what does acidity help with?

A

1) RECYCLING of R’s back TO GOLGI

2) PROCESSING of proteins

91
Q

what is another name for v-snare and what mem is this snare on?

A

synaptobreVin

v = snare on the Vesicle membrane

92
Q

t-snare aka

A

SYNTAXIN

93
Q

what is inhibited in BOTULISM?

A

INHIB of ACh (Excitatory Neurotransmitter)

= FLACCID PARALYSIS

94
Q

what is inhibited in TETANUS?

A

inhib GAB which is an INHIBITORY neuroT thus…
SPASTIC PARALYSIS b/c muscs can’t be inhibited

CLEAVE SYNAPTOBREVIN

95
Q

which SNARE is cleaved in Tetanum & Botulism??

A

synaptobrevin (v-snare)

96
Q

what are the 3 methods of ENDOcytosis and what is unique about them?

A

1) Pinocytosis – no coat needed
2) Phaogcytosis - REQUIRE R. done by macrophages, DCs… & clathirin INDEPENDENT?

3) R-mediated endocytosis -

97
Q

what form(s) of endocytosis do NOT need clathirin

A

pino & phagocytosis

98
Q

What forms of endocytosis do not need CLATHIRIN?

A

PHAGO & PINO

99
Q

What form of endocytosis is used for defense against infection?

A

PHAGO

100
Q

Receptor triggered forms of endocytosis =?

And what is the major difference between them?

A

PHAGO & R-mediated endo

Phago = CLATHIRIN INDEPENDENT

101
Q

Phagosome + lysosome = ____

A

Phagolysosome

102
Q

In what path/process is R-mediated endo WITH CLATHIRIN used for?

A

Tx TRANS-golgi → LYSOSOME 4 degradation

Will have the M6P tag on it (which was done in the cis-golgi)

103
Q

In R-med endocytosis,
Where will these receptors cluster around?

What happens after R is bound?

A

Accumulate in LIPID RAFTS

CLATHRIN binds to receptors

104
Q

Steps of R-med Endocytosis

A

1) Ligand bind 2 receptor @ LIPID RAFT
2) ADAPTIN binds R → RECRUITS CLATHRIN
3) clathrin cause MEM 2 DEFORM
4) DYNAMIN SQUEEZES together
5) clath disassembles
6) RAB-GTP interact RAB-bind P
7) Ves docks when SNARES wind together
8) CIS-SNARE cmplx UNWINDS snares 4 recylcing

105
Q

What allows the mem R to RELEASE CARGO & get RECYCLED

A

↓ pH in ENDOSOME

106
Q

What is the FIRST thing that the ves ALWAYS fuses with after ENDOytosis?

A

EARLY ENDOSOME

107
Q

What is the defect in class IV familial hypercholesterolemia?

What does this defect lead to?

A

LDL-R Unable to CLUSTER
no cluster = no endo = no break↓ of LDL

= ↑ plasma cholesterol & ↑ LDL synth

108
Q

What kind of environment is necessary for proper function of the lysosome?

A

ONLY WORKS @ pH 4.5-5

109
Q

What is the difference between primary & secondary lysosomes?

A 
Primary = NO EXPOSURE to material
Secondary = EXPOSED & broke down material already
110
Q

A vesicle from the early endosome can go to 2 spots next. What are those two spots?

A

1) LATE endosome → Lysosome
OR
2) GOLGI → ER

111
Q

A protein with M6P tag from the cis-Golgi will take what route next?

A

Trans-Golgi → early endosome → late endosome → LYSOSOME

112
Q

Steps in creating golgi -> lysosome vesicle

A

1) cargo R’s bind 2 cargo P’s
2) ADAPTINS LINK R’s to CLATHRIN
3) DYNAMIN pinches off vesicle
4) coating P’s break↓ which EXPOSES SNARES
5) SNARES help with DOCKING

113
Q

T/F Coat proteins are involved in trans-golgi → PM

A

FALSE

114
Q

What DISSOCIATES CARGO from R?

A

ACID / ↓ pH

115
Q

What maintains ↓ pH in lysosome?

A

V-Type ATPase

116
Q

What protects lysosome from digestion from ↓ pH?

A

Mem is HEAVILY GLYCOSYLATED

117
Q

What does a protein need to join multivesicular body?

A

MONOUBIQUITIN TAG

118
Q

What is the purpose of multivesicular bodies?

A

↓ REG of R’s

119
Q

I-Cell diz keywords:

A
  • EMPTY LYSOSOMES
  • ↓ ↓ N-acetylglucosamine phosphotransferase = NO M6P tag

Coarse face, PSYCHOMOTOR RETARD, enlarged liver, ehart

120
Q

Hurler deficiency

A

Alpha-L IDURONIDASE

121
Q

Hunter deficiency

A

Iduronodate sulphatase

Mn: IS → they hunt for people

122
Q

Mutation in Chediak-Higashi syn

A

CHS1/LYST mut → delayed fusion of phagosome with lysosome in LEUKOCYTES

123
Q

Deficiency in METACHROMATIC LEUKODYSTROPHY

A

↓ ARSA

= accum SULFATIDES in cells (toxic to NS) → DESTROY MYELIN

= progressive leukodystrophy in CNS & PNS

Progressive loss of muscle

124
Q

What is the function of peroxisomes??

A

Make H2O2 → 4 oxidation

Have CATALASE → destroy XS h2o2 & free radicals

125
Q

Peroxisomal P’s made in cytosol need what to move INTO peroxisome?

A

SKL import signal binds to PTS1R →
SKL = PTS1R bind PEX (peroxin translocators)
-Need ATP then you an import P

FULLY FOLDED P’s enter

126
Q

PEROXISOMES make PLASMALOGEN. What it this important for?

A

Needed 4 MYELINATION

127
Q

What do peroxisomes break down?

A

VLCFAs (by beta-oxidation)
H2O2
Purines

128
Q

Where are VLCFAs broken ↓ ?

A

Starts Break ↓ in PEROX

Finish in MITO

Can ONLY be broken down in perox

Break↓ of FA= MAJOR nrg source

129
Q

Peroxisomes break ↓ purines.. what is the product?

A

Purine break ↓ into uric acid (XANTHINE OXIDASE)

130
Q

Why does gout occur??

What is the treament and what does. That do?

A

↑ ↑ uric acid → accum of URATE CRYSTALS

Treat with ALLOPURINOL =. Xanthine oxidase inhibitor

131
Q

2 disorders of peroxisome

A

Zellwegers

X-linked adrenoleukodystrophy

132
Q 
Name the disease:
EMPTY PEROXISOMES (b/c don't recog SKL)
VCLFA accumulation (→ ↑ ↑ toxic glial cells)

↓ ↓ PLASMALOGENS

& what are symptoms?

A

Zellwegers

Prominent FOREHEAD, WIDE Eyes (hypertelorism)

133
Q

What are symptoms and dix name?

DEFECT IN TX OF VLCFAs into perox =
no break ↓ 
= accum in: 
1) Brain → accum of toxic glial → myelin breakdown
2) ADRENAL CORTEX → adrenal atrophy
A

x-linked adrenoleukodystrophy

Symp = VISION LOSS, spasticity, ataxia, apathy

LATER ONSET

PROGRESSIVE

134
Q

Where is CARDIOLIPIN made and what does it do?

A

Made in MITO OUTER mem
INSERTED and fn in inner mem

Fn = contributes to PERMEABILITY of inner mem (2 small molecules)

135
Q

ATP synthase is in the INNER mem but ATP is made in ___?

A

MATRIX

136
Q

What is the inner mem of the mito IMPERMEABLE TO?

A

MOST MOLECULES & IONS

137
Q

T/F Majority of proteins that fn inside the mito are ENCODED in the mito

A

FALSE

…in the NUCLEAR GENOME

138
Q

Cardiolipin

How many FA tails?
Where is it made?
Where is it inserted and where does it fn?

A

4 FA tails
Made in OUTER mem
Inserted & fns in INNER mem

139
Q

↓ ↓ cardiolipin leads to

What is the name of this condition?

A

Cardiac failure (↑ mortality in infants)
General musc weakness
Neutropenia

BARTHS syndrome

140
Q

What happens in uncoupling? And why?

What causes this?

A

Inner mem permeable so the protons pumped out by ETC can leak back thru and H+ coming back INTO matrix GENERATES HEAT

THERMOGENIN allows uncoupling/leaking

141
Q

Difference btw intrinsic and extrinsic paths of APOPTOSIS

A

Intrinsic = involves MITO & cytochrome C , BAD/BAX→ caspase 9 → …

Extrinsic = caspase EIGHT → …

Both then → caspase 3/7 → apop

142
Q

Steps of importing P → mito

A

1) P bind HSP70 (prev early folding)
2) HSP70 bind TOM
3) TOM recog mito sig seq
4) HSP70 move thru TIM (pulls into matrix)
5) Matrix protease trims sig seq
6) P binds HSP60 (4 proper fold)

P → HSP70 → TOM → TIM → HSP60

143
Q

What does HSP70 do

A

PREVENTS EARLY FOLDING of p

144
Q

In MITO P import, what RECOG’s MITO SIG SEQ?

A

TOM

145
Q

What COUPLES oxidation with phosphorylation in mito?

A

ATP SYNTHASE

146
Q

What is the role of CASPASE?

A

ALLOWS SCRAMBALASE 2 WORK

Less simplified: it INHIBs P that INHIBS SCRAMBALASE

Scrambalase now works and FLIPS PHOSPHATIDYL SERINE to OUTER LEAFLET during apoptosis

147
Q

Where is most of the mito P’s encoded

A

in NUCLEAR GENOME

148
Q

What is a feature of the H strand of mito DNA?

And light strands?

A

↑ ↑ G’s
Encodes most genes

L-Stranf = ↑ ↑ C’s

149
Q

What is unique about the D-loop in mito DNA?

A

Contains CONTROL REGION, ORIGIN & PROMOTERS

150
Q

Are the strands of mito DNA mono or polycistronic?

A

POLYCISTRONIC

Mn: think that mito evolved from bac which are polycis

151
Q

How many GENES does mito DNA have?
How many are PROTEIN CODING?

tRNA?
rRNA?

A

37 genes – ONLY 13 = P-coding

22 tRNA
2 rRNA

152
Q

T/F mito DNA has histones

A

FALSE

153
Q

What is the name of the pol and helicase for mitoDNA?

A

Gamma pol

Helicase = TWINKLE

154
Q

What does the PLOG gene in mito do?

A

Encodes CATALYTIC SUBUNIT

155
Q

What does a mut in PLOG lead to?

A

↓ [mitoDNA]

Mutation in mtDNA

156
Q

T/F mtDNA has NO okozaki fragments

A

TRUE

157
Q

Characteristics of AZT

A

THYMIDINE ANALOG but has 3 N’s @ 3’ end so STOPS ELONGATION of RT in virus

BUT also FCs Dpol GAMMA → ↓ ↓ mtDNA = myopathy

158
Q

T/f mRNAs in mito are capped

A

FALSE…. not capped

159
Q

Name of diz:

Mut in PLOG or twinkle, late onset, BILATERAL PTOSIS

A

Progressive External Opthalmoplegia

PEO

160
Q

Mito TC:
Where are the promoters in the mtDNA?
What is made?
What is unique in post-TC process?

A

Promoters in D-region
Product = POLYCISTRONIC transcript
-NO CAPPING

161
Q

Mito TL:
What AA does mito TL start with?

Is there more or less wobble than normal?

A

Start with N-FORMYL Methionine … ~ to bacteria

MORE WOBBLE

162
Q

Main differences btw nuclear genome and mito genome (6)

A

Mito genome =

1) LESS genes (37)
2) NO INTRONS
3) NO RECOMBINATION
4) maternal inheritance
5) NO HISTONES
6) FASTER MUTATIONS b/c of free radicals

163
Q

What are the 3 major types of mut’s in mito inheritance

A

Point
Deletion/duplication
Missense

164
Q

How do you define heteroplasmy in mito genes?

A

MIX in normal and mut mito within a cell.
When these get passed on in inheritance, the more the mut mito that get passed on after BOTTLENECK = ↑r chance of diz

But MUST PASS THRESHOLD

165
Q

Severe mutations will be observed in homo or hetero PLASMY?

A

HETERO ONLY

B/c mut in homoplasmy then = FATAL

166
Q

Genetic bottleneck for mtDNA

A

= RANDOM SAMPLING

= # of mut mito inherited is by random chance

167
Q

2 GIANT DELETION of mtDNA diz’s = ?

A

KSS - Kearns-Sayre syn

Pearson syn

168
Q 
What diz?
Giant deletion of mtDNA
WITHOUT bone marrow development
LATE onset
Only FX MUSCS
A

Kearns Sayre Syn

KSS

169
Q

What diiz?
GIANT mtDNA deletion
WITH bone marrow development
FXs ALL TISSUES

A

PEARSON syn

170
Q

What are the 2 diz’s of mito tRNA gene Mutation?

A

MELAS

MERRF

171
Q

What is mutated in this condition?

STROKE like episodes

A

Mut gene = tRNA LEUCINE
Diz = MELAS
Mn: Stroke = melaS
Mn: Leu ~ Lu → Meli → melas

ALWAYS heteroplasmic

172
Q

Which is the ONLY HOMOPLASMIC mito diz that is non-fatal?

A

LHON

173
Q

What is the mutation and the diz?

PROGRESSIVE epilepsy

A

Mut in tRNA LYSINE

Diz= MERRF

174
Q

Which diz’s are cause by what:
GIANT mtDNA DELETIONS?
Mito tRNA mut?
Mito P-coding gene mut?

A

GIANT mtDNA DELETIONS = pearson and kearn sayre

Mito tRNA mut = MERRF & MELAS

Mito P-coding gene mut = LHOM & NARP

175
Q

Mutation in SUBUNITS of COMPLEX I (NADH DEHYDROGENASE)

A

LHON

Bilat visual failure

176
Q

HOMOPLASMIC diz

A

LHON

Mut nadh dehydrogenase (Complex I)

177
Q

What is the diz?

mut ATPase 6 gene of COMPLEX V

A

NARP (musc weak and retinitis pigmentosa)

MCB I (5 decks)

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