scholarly journals Molecular Mechanisms of Cardiac Amyloidosis

2021 ◽  
Vol 23 (1) ◽  
pp. 25
Author(s):  
Yukihiro Saito ◽  
Kazufumi Nakamura ◽  
Hiroshi Ito
Keyword(s):  
Amyloid Precursor Protein ◽  
Cardiac Amyloidosis ◽  
Molecular Mechanisms ◽  
Cardiac Involvement ◽  
Amyloid Deposition ◽  
Precursor Protein ◽  
Systemic Amyloidosis ◽  
Al Amyloidosis ◽  
Attr Amyloidosis ◽  
Cardiovascular Cells

Cardiac involvement has a profound effect on the prognosis of patients with systemic amyloidosis. Therapeutic methods for suppressing the production of causative proteins have been developed for ATTR amyloidosis and AL amyloidosis, which show cardiac involvement, and the prognosis has been improved. However, a method for removing deposited amyloid has not been established. Methods for reducing cytotoxicity caused by amyloid deposition and amyloid precursor protein to protect cardiovascular cells are also needed. In this review, we outline the molecular mechanisms and treatments of cardiac amyloidosis.

scholarly journals The Amyloid Precursor Protein Intracellular Domain-Fe65 Multiprotein Complexes: A Challenge to the Amyloid Hypothesis for Alzheimer's Disease?

2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Daniel A. Bórquez ◽  
Christian González-Billault
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Amyloid Precursor Protein ◽  
Molecular Mechanisms ◽  
Precursor Protein ◽  
Amyloid Peptide ◽  
Intracellular Domain ◽  
Multiprotein Complexes ◽  
Amyloid Cascade

Since its proposal in 1994, the amyloid cascade hypothesis has prevailed as the mainstream research subject on the molecular mechanisms leading to the Alzheimer's disease (AD). Most of the field had been historically based on the role of the different forms of aggregation ofβ-amyloid peptide (Aβ). However, a soluble intracellular fragment termed amyloid precursor protein (APP) intracellular domain (AICD) is produced in conjunction with Aβfragments. This peptide had been shown to be highly toxic in both culture neurons and transgenic mice models. With the advent of this new toxic fragment, the centerpiece for the ethiology of the disease may be changed. This paper discusses the potential role of multiprotein complexes between the AICD and its adapter protein Fe65 and how this could be a potentially important new agent in the neurodegeneration observed in the AD.

scholarly journals Diagnostic approach to light-chain cardiac amyloidosis and its differential diagnosis

2018 ◽  
Vol 49 (1) ◽  
pp. 9-14
Author(s):  
Monika Adamska ◽  
Anna Komosa ◽  
Tatiana Mularek ◽  
Joanna Rupa-Matysek ◽  
Lidia Gil
Keyword(s):  
Differential Diagnosis ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Diagnosis And Treatment ◽  
Diagnostic Approach ◽  
Al Amyloidosis ◽  
Delay In Diagnosis ◽  
Light Chain Amyloidosis ◽  
Novel Approaches

AbstractCardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.

scholarly journals Increased Extracellular Amyloid Deposition and Neurodegeneration in Human Amyloid Precursor Protein Transgenic Mice Deficient in Receptor-Associated Protein

2002 ◽  
Vol 22 (21) ◽  
pp. 9298-9304 ◽  
Author(s):  
Emily Van Uden ◽  
Margaret Mallory ◽  
Isaac Veinbergs ◽  
Michael Alford ◽  
Edward Rockenstein ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Amyloid Precursor Protein ◽  
Amyloid Deposition ◽  
Precursor Protein ◽  
Receptor Associated Protein

scholarly journals Effectiveness of Bortezomib in Cardiac AL Amyloidosis: A Report of Two Cases

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Santi Nigrelli ◽  
Giuseppe Curciarello ◽  
Piercarlo Ballo ◽  
Stefano Michelassi ◽  
Francesco Pizzarelli
Keyword(s):  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Chronic Kidney Disease Stage ◽  
Clinical Results ◽  
Disease Stage ◽  
Comorbid Condition ◽  
Al Amyloidosis ◽  
Therapeutic Approaches ◽  
Hematologic Disease ◽  
Total Remission

Cardiac involvement is a major prognostic determinant in patients with primary AL amyloidosis. The clinical results of standard therapeutic approaches are suboptimal. It has been recently shown that bortezomib, an inhibitor of the proteasome, can induce rapid favourable responses in AL amyloidosis improving cardiac function and survival. Herein we report on two patients with cardiac amyloidosis treated by bortezomib who experienced partial or total remission of hematologic disease and of cardiac involvement. However, death of one patient, suffering from chronic kidney disease stage 5, due to fulminant respiratory syndrome suggests the need for caution in bortezomib use if patients have this comorbid condition.

scholarly journals Early-onset Amyloid Deposition and Cognitive Deficits in Transgenic Mice Expressing a Double Mutant Form of Amyloid Precursor Protein 695

2001 ◽  
Vol 276 (24) ◽  
pp. 21562-21570 ◽  
Author(s):  
M. Azhar Chishti ◽  
Dun-Shen Yang ◽  
Christopher Janus ◽  
Amie L. Phinney ◽  
Patrick Horne ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Amyloid Precursor Protein ◽  
Cognitive Deficits ◽  
Early Onset ◽  
Double Mutant ◽  
Amyloid Deposition ◽  
Precursor Protein ◽  
Mutant Form

scholarly journals Interaction between amyloid precursor protein and Nogo receptors regulates amyloid deposition

2011 ◽  
Vol 25 (9) ◽  
pp. 3146-3156 ◽  
Author(s):  
Xiangdong Zhou ◽  
Xiangyou Hu ◽  
Wanxia He ◽  
Xiaoying Tang ◽  
Qi Shi ◽  
...  
Keyword(s):  
Amyloid Precursor Protein ◽  
Amyloid Deposition ◽  
Precursor Protein ◽  
Nogo Receptors

scholarly journals Neurodegeneration in Alzheimer Disease: Role of Amyloid Precursor Protein and Presenilin 1 Intracellular Signaling

2012 ◽  
Vol 2012 ◽  
pp. 1-13 ◽  
Author(s):  
Mario Nizzari ◽  
Stefano Thellung ◽  
Alessandro Corsaro ◽  
Valentina Villa ◽  
Aldo Pagano ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Amyloid Precursor Protein ◽  
Intracellular Signaling ◽  
Molecular Mechanisms ◽  
Neurodegenerative Disorder ◽  
Precursor Protein ◽  
Presenilin 1 ◽  
Recent Theory ◽  
Intracellular Signals ◽  
Transduction Mechanisms

Alzheimer disease (AD) is a heterogeneous neurodegenerative disorder characterized by (1) progressive loss of synapses and neurons, (2) intracellular neurofibrillary tangles, composed of hyperphosphorylated Tau protein, and (3) amyloid plaques. Genetically, AD is linked to mutations in few proteins amyloid precursor protein (APP) and presenilin 1 and 2 (PS1 and PS2). The molecular mechanisms underlying neurodegeneration in AD as well as the physiological function of APP are not yet known. A recent theory has proposed that APP and PS1 modulate intracellular signals to induce cell-cycle abnormalities responsible for neuronal death and possibly amyloid deposition. This hypothesis is supported by the presence of a complex network of proteins, clearly involved in the regulation of signal transduction mechanisms that interact with both APP and PS1. In this review we discuss the significance of novel finding related to cell-signaling events modulated by APP and PS1 in the development of neurodegeneration.

Sign in / Sign up

Export Citation Format

Share Document