Leucocyte cell-derived chemotaxin 2 related amyloidosis: Difference between revisions
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{{familytree | | | | | | | | | A01 | | | | | |A01= | {{familytree | | | | | | | | | A01 | | | | | | | | | | | | | | B01 | | | | | | | A01=Unidentified Genetic Mutation|B01=Hepatocelluar Damage}} | ||
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{{familytree | | | | | | | | | |`|-|-|-|-|-|-|v|-|-|-|-|-|-|-|-|'| }} | |||
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{{familytree | | | {{familytree | | | | | | | | | | | | | | | | B01 | | | | | |B01= LECT2 overexpression by hepatocytes}} | ||
{{familytree | | | | | | | | | |!| | | | | | | | }} | {{familytree | | | | | | | | | | | | | | | | |!| | | | | | | | | | }} | ||
{{familytree | | | {{familytree | | | | | | | | | | | | | | | | B01 | | | | | |B01= Misfolded LECT2 protein}} | ||
{{familytree | | | {{familytree | | | | | | | | | | | | | | | | |!| | | | | | | | | | }} | ||
{{familytree | | {{familytree | | | | | | | | | | | | | | | | B01 | | | | | |B01=Increased local concentrations <br> Interactions with components of extracellular matrix, such as laminin and type IV collagen <br> Bindings with serum amyloid P (SAP) <br> apoE, and glycosaminoglycans (GAGs)}} | ||
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{{familytree | | {{familytree | | | | | | | | | | | | | | | | B01 | | | | | |B01=Amyloid fibril formation and stabilization.}} | ||
{{familytree | | | | | | | | | | | | | | | | |!| | | | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | | | B01 | | | | | |B01=ALECT2 amyloidosis}} | |||
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Revision as of 17:08, 30 October 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]
Synonyms and keywords:LECT2 amyloidosis
Overview
Historical Perspective
- The first case of ALECT2 was discovered by Benson et al in 2008.
Classification
Pathophysiology
- The pathogenesis of this disease is related to accumulation of a protein called LECT2.
- LECT2 protein is a multi functional factor involved in
- Chemotaxis
- Inflammation
- Immunomodulation
- Damage/repair process.
- LECT2 is synthesized mainly by hepatocytes but also expressed in many organs, including vascular endothelial cells, smooth muscle cells, adipocytes, and epithelial cells such as renal tubular epithelial cells.
- According to the literature, ALECT2 involves G/A polymorphism affecting nucleotide 172 in exon 3 of the LECT2 protein.
- Substitution of the isoleucine with valine at position 40 makes the protein unstable imparting an amyloidogenic property to the LECT2 protein.
- Alternately the disease could be due to interference in the LECT2 catabolic pathway or LECT2 transport.
- Possibly resulting from a genetic defect which ultimately results in an increased local tissue concentration of LECT2 leading to amyloid fibril formation.
- The kidney is the primary target of this disease. '
- Other common organs involved other than the kidney include liver, spleen, prostate, gastrointestinal tract, peripheral nervous system, and lungs.
- Cardiac involvement never occurs, which gives this disease a survival advantage compared to other forms of amyloidosis.
- Other organs which are not involved include brain, pancreas, and fibroadipose tissue.
| Unidentified Genetic Mutation | Hepatocelluar Damage | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| LECT2 overexpression by hepatocytes | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Misfolded LECT2 protein | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Increased local concentrations Interactions with components of extracellular matrix, such as laminin and type IV collagen Bindings with serum amyloid P (SAP) apoE, and glycosaminoglycans (GAGs) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Amyloid fibril formation and stabilization. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| ALECT2 amyloidosis | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||