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Kaplan Qbank USMLE



Author9 Posts
  #1

Familial hypertrophic cardiomyopathy is most likely to be secondary to a mutation in :

a) myosin regulatory proteins
b) myosin binding protein-C
c) Myosin light chains
d) Troponin I
e) Troponin T


___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

  #2

C Myosin light chains

  #3

From UW - AD, mutation in beta-myosin heavy chain

Also for review - what cause dilated CMP and what can cause restrictive CMP?

  #4

I assuming that we talking in reference to the drugs then for
Dilated CMP = Doxorubicin n cocaine & for
Restrictive CMP i think there is not a drug implicated but occurs in Hemochromatosis, AMyloidosis, Pompe's disease

___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

  #5

i didn't know about cocaine - is it for sure?
other ones - antracyclines (daunorubicin, doxorubicin, epirubicin, ida rubicin)
can be prevented by Dextrazoxane - Fe-helating agent.

Restrictive CMP - hemochromatosis, sarcoidosis, amyloidosis, radiation therapy.

  #6

Cocaine is mentioned in Goljan i didnt know that apart from Doxorubicin other drugs of its class r also involved in the Dilated CMP,

___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

  #7

Troponin T

( sorry, light chains now in research, in mice, can also provide CH
But familiar AD will be by heavy and troponin

  #8

this one was a new thing for me too

The correct answer is B

Explanation


Molecular genetic research has demonstrated that familial hypertrophic cardiomyopathy is caused by a mutation in one of nine genes that encode sarcomere proteins. Sarcomeres are the contractile units within the cardiac cells; these are composed of many proteins that are organized into thin and thick filaments. These filaments slide with respect to each other during cardiac contraction.

Mutations in thick filament proteins called cardiac B myosin heavy chain or cardiac myosin binding protein-C appear to account for approximately 82% of hypertrophic cardiomyopathy.

Mutations in thin filament proteins cardiac troponin T and a tropomyosin account for about 13% of hypertrophic cardiomyopathy.

Mutations in two other sarcomere proteins, the myosin regulatory and essential light chains are quite rare and contribute less than 5% to hypertrophic cardiomyopathy.

Although these percentages are only estimates, they indicate that more disease-causing genes will be identified. A tenth gene has been identified that is a non-sarcomere protein (a subunit of protein kinase A) is associated with individuals who have both hypertrophic cardiomyopathy and Wolf-Parkinson-White syndrome.




___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."

  #9

Very good, Now it's clear why it's a confused but a good Q. I feel that there are many ans, did not know about this %. Good research, thank you.
I am still with pharma...







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