Seidler Contractile Proteins Flashcards

1
Q

MyHC IIa gene and muscle fiber type?

A

MYH2

type 2A

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2
Q

embryonic MyHC gene and muscle fiber type?

A

MYH3

fetal development, muscle regeneration

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3
Q

Smooth Muscle MyHC gene and muscle fiber type?

A

MYH11

smooth m.

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4
Q

fetal MyHC gene and muscle fiber type?

A

MYH8

fetal development, muscle regeneration

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5
Q

Beta-cardiac MyHC gene and muscle fiber type?

A

MYH7

heart ventricles

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6
Q

alpha-cardiac MyHC gene and muscle fiber type?

A

MYH6

heart atria

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7
Q

Extraocular MyHC gene and muscle fiber type?

A

MYH13

extraocular muscle

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8
Q

MyHC 1 gene and muscle fiber type?

A

MYH7

type 1

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9
Q

mutation in what is associated with myosin storage myopathy?

what kind of myopathy?

A

MYH7

distal rod

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10
Q

where does myosin self-assemble?

what chains make it up?

what can the head region do?

what is its rotational symmetry?

A

at the tails

2 heavy, 4 light

hydrolyze ATP

30 degrees

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11
Q

how is actin arranged?

A

G-actin assembles into F-actin

Each G-actin binds 4 neighbors

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12
Q

what binds free G-actin first?

A

ATP

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13
Q

what does tropomodulin do?

A

caps the minus end, preventing further polymerization

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14
Q

what does CapZ (b-actinin) do?

A

associates w/a-actinin

caps at the plus end

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15
Q

what does Nebulin do?

A

ruler and template for actin polymerization

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16
Q

what does Troponin T bind to?

function?

A

tropomyosin

positions the complex on the filament

17
Q

what does Troponin I bind to?

function?

A

actin

inhibits myosin binding

18
Q

what does Troponin C bind to?

function?

A

binds Ca2+

relieves inhibition

19
Q

what do costameres do?

A

tether Z disc to sarcolemma

20
Q

what is titin?

functions?

A

sarcomere ruler

passive elasticity
muscle signaling
balances forces across sarcomere

21
Q

what is diagnostic for a heart attack?

22
Q

describe the smooth m. contraction with Calcium and calmodulin

A

Calmodulin binds calcium, this binds the myosin light-chain kinase
kinase will phosphorylate the myosin light chain and myosin proteins get activated

23
Q

where is dystrophin located?

function?

A

costameres

tethers myofiber bundles to surface membranes and maintains the integrity of surface membrane during sheer stress of muscle contraction

24
Q

What is GSD V/McArdle disease?

A

deficiency in MUSCLE glycogen phosphorylase (RLS of glycogen degradation to release G1P)

25
what are the Sx of McArdle disease?
m. cramping, myoglobinuria it is allosterically activated by AMP
26
What is Her's disease? how is it regulated?
GSD VI, mutation in LIVER glycogen phosphorylase allosterically inhibited by ATP and G6P inactivated by free glucose, NOT AMP
27
what is sensitive to calcium?
muscle isozyme of phosphorylase
28
defect in dystrophin results in what?
DMD, costamere disorganization, sarcolemma fragility, muscle weakness
29
what is common with in-frame dystrophin mutations? out-frame?
BMD DMD
30
what is found in cardiac muscle after a MI? what is found in skeletal muscle?
cTn-1 sTn-1
31
MyHC IIx/d gene and muscle fiber type?
MYH1 type 2b