report that endothelial cell-derived endothelin-1 activates mesangial cells, which deposit abnormal laminin isoforms in the Alport glomerular basement membrane.
Two other types of deposits are abnormal versions of proteins involved in other cell functions.
The doctors there found that the patient had a primary form of amyloidosis and that it had spread throughout his body, with the deposits of abnormal protein affecting many of his organs.
Protein aggregation and deposits of abnormal proteins are hallmarks of several neurodegenerative diseases [1].
Immunofluorescence revealed no immune deposits, but abnormal expression of nephrin and podocin was detected in the glomeruli.
LOXL1-deficient mice are viable, but they have deposits of abnormal elastic fibres in the uterine tract postpartum, and they develop pelvic organ prolapse, enlarged airspaces in the lung, loose skin and vascular abnormalities with concomitant tropoelastin accumulation (12).
Bovine Amyloidotic Spongiform Encephalopathy (BASE) was the first of these novel cattle prion disorders to be recognized and was characterized by the presence of numerous small amyloid deposits of abnormal PrP.
Having observed conspicuous filamentous deposits of abnormal PrP in the white matter in autopsy material from patients with inherited prion disease attending the National Prion Clinic and examined at autopsy between 2004 and 2012, we have subsequently analysed our cases systematically for the presence of white matter PrP deposits.
Pathologically, chronic traumatic encephalopathy produces atrophy of the frontal and temporal lobes, thalamus, and hypothalamus; septal abnormalities; and abnormal deposits of hyperphosphorylated tau as neurofibrillary tangles and disordered neurites throughout the brain.
In these processes, growth factors, cytokines, and other mediators are released, followed by excessive extracellular matrix deposit and abnormal mesenchymal cell proliferation in the lungs.
