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Dr. Albert Mihály (2005)
Amyloidosis
Amyloidosis is not a single disease, but adiverse group of
diseases all characterized
by extracellular deposition of ultrastucturally similar,but
biochemically distinct protein.
Amyloid starchlikePathogenesis of amyloidosis isdifferent.
There are two major and several minor bichemical forms.
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Dr. Albert Mihály (2005)
Amyloidosis
Physical nature of amyloidRigid nonbranching fibrils
(EM)Indeterminate lenghtDiameter (approx.): 7,5-10 nmβ-pleated
sheet configurationHelix form with 100 nm periodicityTwo twisted
β-pleated sheet micelles
amyloid fibrils are resistent to proteolytic digestion
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Dr. Albert Mihály (2005)
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Dr. Albert Mihály (2005)
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Dr. Albert Mihály (2005)
Amyloidosis
Detection:Congo-red red
green birefringence (polarized light)iodine solution: brown
Rinsed with 5% sulfuric acid blueViolet dyes red
(metachromasia)Toluidin blue redFluorochromes (thioflavin T)
yellowish yellowish greengreen
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Dr. Albert Mihály (2005)Polarized light
Congo-red
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Dr. Albert Mihály (2005)
Violet dye Iodine solution
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Dr. Albert Mihály (2005)
Amyloidosis
Chemical natureFibril protein 95%P-component 8 nm in diam.
pentagonal glycoprotein (5%)
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Dr. Albert Mihály (2005)
Amyloidosis
Type of amyloid Precursor
AA serum amyloid A(SAA)AL immunoglob. light chainsAI
apolipoproteinIAPP islet amyloid polypeptideSenile β -protein
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Dr. Albert Mihály (2005)
Proposed schema of the pathogenesis of two major forms of
amyloidosis
Stimulus unknown chr. Inflammation
monoclonal B-lymph. Macrophage activationproliferation
interleukin 1 and 6plasma cells
liver cells
Soluble precursors immunglob. Light Chains SAA protein
limited proteolysisInsoluble fibrils AL protein AA protein
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Dr. Albert Mihály (2005)
Amyloidosis
Forms of AmyloidosisSystemic: liver, spleen, kidney, adrenal
gland, intestine (mainly)Localized
Nasal Islet (pancreas)Pulmonary Thyroid (C-cell tumor)Brain
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Dr. Albert Mihály (2005)
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Dr. Albert Mihály (2005)
Cut surface of the liver
Cut surface of the spleen (sago spleen)
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Dr. Albert Mihály (2005)
Amyloidosis, liver
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Dr. Albert Mihály (2005)
Amyloidosis;
intestine
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Dr. Albert Mihály (2005)Amyloidosis; liver, goose
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Dr. Albert Mihály (2005)
Amyloidosis;
nasal septum, horse
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Dr. Albert Mihály (2005)
Islet amyloidosis (cat)
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Dr. Albert Mihály (2005)
Harmful effects of amyloidosis
Funcional disturbanceKindney failureLiver failureDiabetes
…..etc.
Haemorrhage due to rupture of organs
AmyloidosisAmyloidosisAmyloidosisAmyloidosisAmyloidosisProposed
schema of the pathogenesis of two major forms of
amyloidosisAmyloidosisHarmful effects of amyloidosis
