OBJECTIVE To clarify the clinical significance of the SAA1.3 allele in the development and outcome of AA amyloidosis in Japanese patients with rheumatoid arthritis (RA). METHODS One hundred and twenty RA patients (60 alive and 60 dead) fulfilling the 1987 ACR criteria and 62 RA patients with biopsy-confirmed amyloid A (AA) amyloidosis (36 alive and 26 dead) were enrolled. The SAA1 genotypes w...

protein aggregation is a serious problem for both biotechnology and cell biology. diseases such as prion misfolding, alzheimer’s, and other amyloidosis are phenomena for which protein aggregation in our living cells is of considerable relevance. human lysozyme has been shown to form amyloid fibrils in individuals suffering from nonneuropathic systemic amyloidosis, all of which have point mutati...

Protein misfolding and aggregation are thought to underlie the pathogenesis of many amyloid diseases, such as Alzheimer and Parkinson diseases, whereby a stepwise protein misfolding process begins with the conversion of soluble protein monomers to prefibrillar oligomers and progresses to the formation of insoluble amyloid fibrils. Drusen are extracellular deposits found in aging eyes and in eye...

BACKGROUND Systemic amyloidoses include immunoglobulin light chain (AL) amyloidosis, serum amyloid (AA)-related amyloidosis and senile systemic amyloidosis (SSA). AL amyloidosis is associated with myeloma, and we showed recently that transthyretin-related hereditary amyloidosis was related to non-Hodgkin lymphoma (NHL). In SSA, amyloids constitute wild-type transthyretin. We wanted to analyze c...

Introduction: Because of bilateral involvement in many cases with diabetic macular edema, Intravitreal bevacizumab injection perform in both eyes. There are some reports of therapeutic effects in the fellow eye after monocular injection of anti-vascular endothelial growth factors. In this study we describe a case of bilateral improvement of diabetic macular edema following unil...

The amyloidoses are a group of protein-misfolding disorders characterized by the accumulation of amyloid fibrils formed from a variety of proteins. Currently, twentyeight different kinds of human and animal proteins, in intact or fragmented forms, have been found to be associated with pathological disorders such as Alzheimer’s disease, type II diabetes, prion diseases, dialysis-related amyloido...

The median survival in primary systemic (AL) amyloidosis is less than 18 months. No published series of patients with AL amyloidosis have reported survival of more than 10 years. The records of all Mayo Clinic patients with a diagnosis of AL amyloidosis between January 1, 1966 and March 1, 1987 were reviewed. Patients with secondary amyloidosis, familial amyloidosis, senile systemic amyloidosis...

Amyloidosis is a rare, mysterious and serious disorder. The extracellular deposition of seemingly homogenous and amorphous material actually represents the expression of very diverse pathologies. All amyloid fibrils share apple green birefringence under polarized light with Congo-red staining, and are arranged in beta-pleated structure on electron microscopy. More than 21 proteins have been ide...

In immunoglobulin light-chain (L) amyloidosis the cause of death is most commonly due to cardiac involvement. Cardiac amyloidosis is frequently diagnosed by postmortem examination. There are many reports of diagnoses of cardiac amyloidosis established by autopsy of cardiac tissue. We report four patients whom we could diagnose as having cardiac amyloidosis by echocardiogram and electrocardiogra...

There are two major forms of amyloidosis, primary amyloidosis (AL) and secondary amyloidosis. AL amyloidosis results from deposition of immunoglobulin light chains or their fragments. One such example is AL amyloidosis associated with multiple myeloma, in which overproduced immunoglobulin light chains get deposited onto tissues, leading to tissue dysfunction. Amyloidosis in the intestines can p...