GMS 6474 Study Guide Questions with Verified
Correct Answers
HOw many types of MD are known?
more than 20 different types of MD that are known
Ducheene Muscular Dystrophy is what?
An X-linked disease caused by a mutation in dystrophin
True/false: Dystrophin is one of the largest genes in the human body
TRUE!
79 exons and 2.5 Mb
what happens in the mutation of a dystrophin gene?
absence of partly functioning dystrophin protein
True/false: sarcolemma has alot of strain due to the myofibrils acting on it
true
name the 7 glycoproteins that in the DAC?
a,b - DG
a,b,g,l - SG
sarcospan
what is sarcospan for?
connects the DAC to dystrophin
Domain 1 and 2 on the N-terminal ends on dystrophin are what?
actin binding domains
,what is the rod domain composed of?
24 spectrin (r) and 4 hinges (h)
domain 3 is for what?
dystroglycan-binding domain
domain 4?
carboxyl terminal domain, helps to adhere nNOS to subsarcolemmal space
know the pathophysiology of DMD
how many boys inherit this disease?
1:3500
what is gower's signs?
weakness of knee and hip extensors - pt is unable to stand up
know the 2 allelic dystrophinopathies
review 15:00
prednisone and deflazacort do what?
decrease inflammation and increase ampulation by 1 year
how doe PT help?
maintains muscle flexibility
what are eccentric/pilometric?
regeneration is influenced by activity
satellite cells adds nuclei and muscle fibers increase in size
, you can tell that a muscle is regenerating how?
from the multiple nuclei's
Lecture 10
what is the role of the CV system?
delivers material to body
what is the goal of the CV system?
maintains characteristics of interstitial fluid, plasma, and intercellular compartments
consistency of interstitial fluid is maintained if?
1. blood flow is adequate
2. blood composition is appropraite
3. diffusion distances are short
what % of body weight is water?
60%
of this 60, how much is IC and how much is EC?
2/3 is intracellular and 1/3 is EC
intracellular compartment is how many liters?
30
interstitial compartment is how many liters?
12
plasma is how many liters?
Correct Answers
HOw many types of MD are known?
more than 20 different types of MD that are known
Ducheene Muscular Dystrophy is what?
An X-linked disease caused by a mutation in dystrophin
True/false: Dystrophin is one of the largest genes in the human body
TRUE!
79 exons and 2.5 Mb
what happens in the mutation of a dystrophin gene?
absence of partly functioning dystrophin protein
True/false: sarcolemma has alot of strain due to the myofibrils acting on it
true
name the 7 glycoproteins that in the DAC?
a,b - DG
a,b,g,l - SG
sarcospan
what is sarcospan for?
connects the DAC to dystrophin
Domain 1 and 2 on the N-terminal ends on dystrophin are what?
actin binding domains
,what is the rod domain composed of?
24 spectrin (r) and 4 hinges (h)
domain 3 is for what?
dystroglycan-binding domain
domain 4?
carboxyl terminal domain, helps to adhere nNOS to subsarcolemmal space
know the pathophysiology of DMD
how many boys inherit this disease?
1:3500
what is gower's signs?
weakness of knee and hip extensors - pt is unable to stand up
know the 2 allelic dystrophinopathies
review 15:00
prednisone and deflazacort do what?
decrease inflammation and increase ampulation by 1 year
how doe PT help?
maintains muscle flexibility
what are eccentric/pilometric?
regeneration is influenced by activity
satellite cells adds nuclei and muscle fibers increase in size
, you can tell that a muscle is regenerating how?
from the multiple nuclei's
Lecture 10
what is the role of the CV system?
delivers material to body
what is the goal of the CV system?
maintains characteristics of interstitial fluid, plasma, and intercellular compartments
consistency of interstitial fluid is maintained if?
1. blood flow is adequate
2. blood composition is appropraite
3. diffusion distances are short
what % of body weight is water?
60%
of this 60, how much is IC and how much is EC?
2/3 is intracellular and 1/3 is EC
intracellular compartment is how many liters?
30
interstitial compartment is how many liters?
12
plasma is how many liters?
