Classification of amyloid
What are the different types of amyloid?
Types of amyloidosis include:
- AL amyloidosis (immunoglobulin light chain amyloidosis). This is the most common type of amyloidosis in developed countries. …
- AA amyloidosis. This type is also known as secondary amyloidosis. …
- Hereditary amyloidosis (familial amyloidosis). …
- Wild-type amyloidosis. …
- Localized amyloidosis.
How many amyloids are there?
To date, 37 human proteins have been found to form amyloid in pathology and be associated with well-defined diseases.
What is the difference between primary and secondary amyloidosis?
Secondary means it occurs because of another disease or situation. For example, this condition usually occurs due to long-term (chronic) infection or inflammation. In contrast, primary amyloidosis means there is no other disease that is causing the condition. Systemic means that the disease affects the entire body.
What are the different compositions of amyloid?
Table 1
| Disease | Precursor protein | Amyloid protein |
|---|---|---|
| Atrial amyloidosis | Atrial natriuretic factor (ANF) | Amyloid ANF |
| Spongiform encephalopathies | Prion protein (PrPc) | PrPsc |
| Primary systemic amyloidosis | Immunoglobulin light and heavy chains | AL and AH |
| Senile systemic amyloidosis | Wild-type transthyretin | ATTR |
What is the function of amyloid?
Little is known about the function of amyloid precursor protein. Researchers speculate that it may bind to other proteins on the surface of cells or help cells attach to one another. Studies suggest that in the brain, it helps direct the movement (migration) of nerve cells (neurons) during early development.
What proteins make amyloid?
Secondary amyloid consists of the protein AA (amyloid A) an 8.5 kDa protein that is heterogeneous (at the amino terminal end). It is putatively derived by proteolysis from the normal circulating protein apoSAA (serum amyloid A related protein) which has a molecular weight of 12 kDa.
How many types of amyloid fibrils are there?
To date, 36 distinct proteins have been identified as amyloid fibril proteins in human ( Table 1 ).
What is the difference between localized and systemic amyloidosis?
In systemic amyloidosis, the heart, kidneys, and nerves are most commonly affected, resulting in congestive heart failure, arrhythmia, nephrotic syndrome, renal failure, and peripheral and autonomic neuropathies. In localized amyloidosis, amyloid deposits at the site of production, so only one organ is disrupted.
What are beta-amyloid proteins?
The amyloid-beta precursor protein is an important example. It is a large membrane protein that normally plays an essential role in neural growth and repair. However, later in life, a corrupted form can destroy nerve cells, leading to the loss of thought and memory in Alzheimer’s disease.
What is the most common type of amyloidosis?
AL amyloidosis (immunoglobulin light chain amyloidosis).
This is the most common type and used to be called primary amyloidosis. AL stands for “amyloid light chains,” which is the type of protein responsible for the condition.
What is the life expectancy of a person with amyloidosis?
Average life expectancy varies based on the type of cardiac amyloid (protein), how much the organs are involved and the stage at diagnosis. Based on these factors, the worst case scenario could be six months, while in some cases, life expectancy can be eight to 10 years after diagnosis.
What are amyloids in the brain?
Amyloid plaques are aggregates of misfolded proteins that form in the spaces between nerve cells. These abnormally configured proteins are thought to play a central role in Alzheimer’s disease. The amyloid plaques first develop in the areas of the brain concerned with memory and other cognitive functions.
Where is amyloid found?
Amyloid is an abnormal protein that is produced in your bone marrow and can be deposited in any tissue or organ. Amyloidosis frequently affects the heart, kidneys, liver, spleen, nervous system and digestive tract.
What is end stage amyloidosis?
Cardiac amyloidosis is a condition where faulty proteins build up in your heart. You can inherit this condition, or it can develop on its own (usually later in life). As faulty proteins accumulate, your heart struggles to pump, ultimately leading to heart failure and death.
What is the latest treatment for amyloidosis?
Recent studies have shown that people with newly diagnosed AL amyloidosis, the four-drug combination of subcutaneous daratumumab, bortezomib, cyclophosphamide, and dexamethasone is safe and effective. This treatment is now considered standard of care for most patients.