Leucocyte cell-derived chemotaxin 2 related amyloidosis
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.