If Diffusional Slows Down The Amyloids , How Long Does It Start To Work,I Heard About 14 Months. Is That So
You can have wild type ATTR(wtATTR) but not have cardiomyopathy if it has not infiltrated into your heart.
ATTR-CM is when your disassociated TTR proteins have been deposited extracellularly in and around your heart muscle and have affected t your heart functions.
Extracellular amyloid fibril deposits causes thickening of your heart That will eventually cause stiff heart syndrome (systolic dysfunction) which is a lessening of your heart's ability to pump blood. Also, the amyloid fibrils (broken-up or disassociated TTT proteins) that are misfolded monomers) are proteotoxic.
That means they interfere with the normal functioning of your heart muscle cells. Arythmias such as Atrial flutter or fibrillation are among the manifestations of that type of damage caused by the misfolded TTR. The fibrils can cause electrical conductivity blockages that slow down conduction of the electrical signals to your ventricles (that cause contraction/pumping of blood to your body).
The tetrameric TTR structure is a 4-protein structure. It has a covalent bond with hydrogen atoms at its core (covalent means they share electrons ). When that covalent bond is disrupted, the 4 proteins break apart into 4 separate single proteins or monomers, that no longer fold properly. That is what may lead to amyloid fibrils being formed. Those then can clump (deposit/build up) extracellularly around nerves and organs.
Basically, the broken up proteins can go anywhere that your blood can transport them in your body. That leads to neuropathy, heart damage, kidney damage, etc.,when healthy cells are damaged by the amyloid fibrils toxicity
@A MyAmyloidosisTeam Member just wanted to make sure my question above reached you.
Hi Sheldon. Can the electrical conductivity blockages that slow down conduction cause heart attack or other symptoms that lead to such damage to the heart?
thank you
Hi Sheldon
Is there a difference between attr-cm and wild type attr ?
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