Amyloidosis is the term used for a group of diseases characterized by extracellular deposition of insoluble protienaceous substance called “Amyloid”.Amyloidosis was first described by Rokintansy in 1842. Virchow named it as amyloid under the mistaken belief that the material was starch like.

1. BY Nakka Varshitha
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2.  Definition
 History
 Properties of amyloid protein
 Pathogenesis
 Classification
 Symptoms
 Diagnosis
 treatment
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3. DEFINITION
 Amyloidosis is the term used for
a group of diseases characterized
by extracellular deposition of
insoluble protienaceous
substance called “Amyloid”
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4. HISTORY
 Amyloidosis was first described
by Rokintansy in 1842.
 Virchow named it as amyloid
under the mistaken belief that the
material was starch like.
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5. PHYSICAL PROPERTIES:
 By electron microscope amyloid
is seen to be made up largely of
continous,non branching fibrils
with a diameter of 7.5 to 10nm
 X-ray crystallography and
infrared spectroscopy
demonstrate a characteristics
cross β-pleated sheet
conformation.
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6. CHEMICAL PROPERTIES:
Based on analysis amyloid
composed of two main complex
proteins,
1. Fibril proteins (95%)
2. Non –fibril components (5%)
Fibril proteins
 They consist of delicate,
randomly dispersed, on branching
amyloid fibers having 4-6 fibrils
each measuring 7.5-10nm in
diameter and having indefinite
length.
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7. CHEMICAL PROPERTIES
Non-fibril components:
It contributes 5% of total
amyloid and mainly consists of
following components
1.Amyloid p components
2.Apoprotein-E
3.Sulfated glycosaminoglycans
4.Other components
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8. PATHOGENESIS
The cells in the body have two different ways of making proteins
some proteins are made up of single sequence of aminoacids in other
cases proteins fragments undergoes folding to form the whole protein.
protein components undergoes misfolding
These misfolded proteins forms amyloid precursor proteins called
amyloidgenic monomers which then aggregate to form oligomers
Oligomers aggregate to form amyloid fibrils ,they accumulate in various parts
of the body and causes amylodiosis.
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9. CLASSIFICATION
Amyloidosis has been classified in to number of ways based on
1.CAUSE:
 Primary: it is with unknown cause and it is due to disorder of
immune cell function.
 Secondary: it is by complication of some underlying known disease
2.EXTENT OF AMYLOID DEPOSITION:
 Systemic: it involves multiple organs
 Localised:involves one or two organs
3.CLINICAL SITES:
 Pattern-1: involving tongue, heart, bowel, skeletal and smooth muscle skin
and nerves.
 Pattern-2:involving liver,spleen,kidneyand adrenal
 Mixed pattern
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10. CLASSIFICATION
4. PRECURSOR BIOCHEMICAL PROTEINS
• Primary
• Secondary
• Haemodialysis
• Heredofamilial
SYSTEMIC
AMYLOIDOSIS
• Sensile cardiac
• Sensile cerebral
• Endocrine
• Tumor forming
LOCALISED
AMYLOIDOSIS
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11. SYMPTOMS
The symptoms of amyloidosis are
as follows
• Change in skin color
• Clay coloured stools
• Fatigue
• Feeling of fullness
• Joint pain
• Anaemia
• Shortness of breath
• Swelling of tounge
• Weak hand grip
• Weakness
• Weight loss
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12.  Tissue diagnosis
 congo red test
 protein electrophoresis
 Immunoelectrophories of
urine serum and bone marrow
aspiration
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13.  There is no cure for amyloidosis
 By using treatment to suppress the
development of amyloid forming
protein and to mamage symptoms.
 High dose chemotherapy with stem
cell transplant can help to remove the
substance that leads to amyloid
formation in those with primary AL
amyloidosis who have no more than
two major organ damage
 transplantations may stop the disease
in those with hereditary amyloidosis.
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