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Synthesis of Critical Topics in Alzheimer’s Disease *
Published in Zaven S. Khachaturian, Teresa S. Radebaugh, Alzheimer’s Disease, 2019
Zaven S. Khachaturian, Teresa S. Radebaugh
Amyloid protein has the unusual characteristics of being highly insoluble and resistant to degradation, thus readily accumulating within the nervous system. How it interferes with cell functioning is not totally clear, but there are some suggestions that aggregations of beta-amyloid become highly toxic in neurons in a way similar to glutamate. In fact, both of these substances may inflict their damage by disrupting the internal homeostasis of calcium ions. Recent discoveries concerning the nature of amyloid protein, how it is formed and processed, what it does to a cell, and the genes that determine its structure have created tremendous excitement among neurosci-entists. Many believe that pursuit of this clue will lead to discovery of the specific causes of AD. The chapter by Drs. Suzanne S. Mirra, William R. Markesbery, Juan C. Troncoso, Barbara J. Crain, Sangram S. Sisodia, and Donald L. Price in the section on Biological Markers provides a more detailed overview of the neuropathological lesions and other abnormalities associated with AD.
Musculoskeletal disorders and connective tissue disorders
Published in Steve Hannigan, Inherited Metabolic Diseases: A Guide to 100 Conditions, 2018
The different forms of hereditary amyloidosis do vary with regard to the organs of the body that are involved. Sometimes a single organ may be predominantly affected, while in other forms many organs can be affected. For example, hereditary trans-thyretin amyloidosis mainly afects the nerves, whereas hereditary ibrinogen amyloidosis mainly affects the kidneys. Symptoms therefore vary widely and depend upon which tissues and organs are damaged by the build-up of amyloid protein in each case.
Herbal Medicines in Neuropsychiatric Illness: The Case of L-Stepholidine
Published in Vikas Kumar, Addepalli Veeranjaneyulu, Herbs for Diabetes and Neurological Disease Management, 2018
Nitin Sati, Kedar S. Prabhavalkr, Sridhar Natesan, Lokesh K. Bhatt
Uncaria rhynchophylla (Miq.) Jack and Gastrodia elata BI. are traditional Chinese herbs that are usually used in combination to treat convulsive disorders, such as epilepsy, in China. Anti-convulsive effect of the mentioned herbs was evaluated using in vitro and in vivo studies. The study was carried out with either of the plants and the combination, separately. The results of the study indicated that U. rhynchophylla (Miq.) possesses anti-convulsive activity and its effect is synergized by G. elata BI. U. rhynchophylla has also been used to relieve various neurological symptoms. The methanolic extract of U. rhynchophylla also exhibited neuroprotective activity. Inhibition of β-amyloid protein generation, prevention of β-amyloid protein fibril formation or destabilization of β-amyloid protein may serve as attractive targets for treatment of Alzheimer’s disease. The study revealed that U. rhynchophylla has remarkably inhibitory effects on the regulation of β-amyloid protein fibrils, and thus could have the potency to be a novel therapeutic agent to prevent and/or cure Alzheimer’s disease.3
Bad players in AL amyloidosis in the current era of treatment
Published in Expert Review of Hematology, 2023
Natalia Kreiniz, Morie A. Gertz
In 2021, the combination of daratumumab, bortezomib, cyclophosphamide, and dexamethasone (DaraCyBorD) was approved by FDA and EMA for the treatment of AL, based on the results of phase 3 clinical trial-ANDROMEDA, which demonstrated higher rate of hematologic complete response and survival free from major organ from major organ deterioration or hematologic progression [11]. DaraCyBorD is currently the only one approved treatment regimen for ALA. Patients with stage IIIB ALA were not included in ANDROMEDA study and represent the most vulnerable subgroup of patients with unmet need for treatment options. The development of new treatment strategies, targeting misfolded amyloid protein depositions in organs is promising for this group of patients and has already demonstrated benefits in clinical trials. Thus, birtamimab improved survival of Mayo stage IV amyloidosis patients with cardiac involvement in the VITAL phase 3 study [12] and Cael-101 demonstrated safety and tolerability in a phase 2 clinical trial [12,13]. Phase 3 trials are ongoing to validate these results [14,15]. Other molecules targeting clonal plasma cells or amyloid are in different stages of development [16]. First reports on the use of anti-BCMA CAR-T cells for the treatment of ALA have been published [17,18].
Lacrimal gland extranodal marginal zone B-cell lymphoma in the presence of amyloidosis
Published in Orbit, 2022
Chung Shen Chean, Vishakha Sovani, Ali Boden, Christopher Knapp
Amyloidosis is a disorder characterised by extracellular deposition of insoluble fibrils with a β-sheet structure derived from aggregation of misfolded proteins. More than 20 types of amyloid proteins have been identified in various human organs.1 The most common and severe form of systemic amyloidosis is the amyloid light chain (AL) amyloidosis.5 The protein consists of filaments of a monoclonal immunoglobulin light chain, typically originating from a small plasma cell clone with isolated monoclonal paraproteinaemia or asymptomatic myeloma. Its association with systemic myeloma or B-cell lymphoproliferative disorders is uncommon.2,5 Laboratory findings include elevated levels of serum immunoglobulin light chain kappa or lambda and Bence Jones protein in the urine.2,9 Diagnosis of amyloidosis is confirmed by staining a biopsy specimen with Congo red stain, which gives a characteristic apple-green birefringent pattern under polarised light.4 Immunocytochemistry can also be useful in identifying the amyloid protein.10
AL amyloidosis presenting as inflammatory polyarthritis: a case report
Published in Modern Rheumatology Case Reports, 2021
Muhammad Shoaib Momen Majumder, Shamim Ahmed, Md. Nahiduzzamane Shazzad, Mohammad Mamun Khan, Syed Atiqul Haq, Mohammed Kamal, Md. Sohrab Alam, Johannes J. Rasker
Amyloid protein can deposit in various organs/tissues. Clinical features depend upon the type of amyloid protein and organ/tissue where deposited. Systemic AL amyloidosis can involve any organ except the central nervous system [2]. An updated definition of organ involvement in AL amyloidosis has been made in a meeting of the International Society of Amyloidosis held in 2010 in Rome [19,20]. Amyloid arthropathy is a relatively uncommon presentation (approximately 5%) of systemic amyloidosis [21]. It is low grade, subacute, progressive, and symmetric with a predilection for shoulders, knees, wrists, MCPs, PIPs, and less commonly elbows and hips. There may be pain, swelling, and morning stiffness that may mimic chronic inflammatory arthropathies [1]. Though amyloid arthropathy is rather uncommon as a presenting feature of amyloidosis, when present, it is regarded as a disease-specific finding in AL amyloidosis [21]. It may also cause nodular hypertrophy of synovium due to amyloid deposits. Rarely erosive arthritis has been reported in patients with AL amyloidosis coexisting with monoclonal gammopathy of uncertain significance [22]. Swelling may be particularly prominent around glenohumeral joints resulting in a characteristic “shoulder pad” sign. It is pathognomonic for AL amyloidosis [23].