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Mass spectrometry-based proteomic analysis of proteins adsorbed by hexadecyl-immobilized cellulose bead column for the treatment of dialysis-related amyloidosis.
Amyloid
June 2024
Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Background: Dialysis-related amyloidosis (DRA) is a severe complication in end-stage kidney disease (ESKD) patients undergoing long-term dialysis treatment, characterized by the deposition of β-microglobulin-related amyloids (Aβ2M amyloid). To inhibit DRA progression, hexadecyl-immobilized cellulose bead (HICB) columns are employed to adsorb circulating β-microglobulin (β2M). However, it is possible that the HICB also adsorbs other molecules involved in amyloidogenesis.
View Article and Find Full Text PDFHuman wild-type and D76N β-microglobulin variants are significant proteotoxic and metabolic stressors for transgenic .
FASEB Bioadv
November 2023
Department of Molecular Medicine, Institute of Biochemistry University of Pavia Pavia Italy.
β-microglobulin (β-m) is a plasma protein derived from physiological shedding of the class I major histocompatibility complex (MHCI), causing human systemic amyloidosis either due to persistently high concentrations of the wild-type (WT) protein in hemodialyzed patients, or in presence of mutations, such as D76N β-m, which favor protein deposition in the adulthood, despite normal plasma levels. Here we describe a new transgenic Caenorhabditis elegans () strain expressing human WT β-m at high concentrations, mimicking the condition that underlies dialysis-related amyloidosis (DRA) and we compare it to a previously established strain expressing the highly amyloidogenic D76N β-m at lower concentrations. Both strains exhibit behavioral defects, the severity of which correlates with β-m levels rather than with the presence of mutations, being more pronounced in WT β-m worms.
View Article and Find Full Text PDFLenalidomide plus Dexamethasone Combination as First-Line Oral Therapy of Multiple Myeloma Patients: A Unicentric Real-Life Study.
Cancers (Basel)
August 2023
Division of Hematology with BMT, A.O.U. Policlinico "G.Rodolico-San Marco", 95123 Catania, Italy.
Based on the results obtained in clinical trials, the use of the combination of lenalidomide and dexamethasone (Len/Dex) has become a potential therapeutic choice for newly diagnosed multiple myeloma (NDMM) ineligible for autologous stem cell transplantation. This study evaluated 89 frail NDMM patients treated with first-line oral association. At the last follow-up, 34 out of 89 patients (38.
View Article and Find Full Text PDFSurgical Treatment of Ischial Ulcers Associated with Deposition of β2-Microglobulin in Two Cases of Dialysis-related Amyloidosis.
Plast Reconstr Surg Glob Open
June 2023
Department of Plastic and Reconstructive Surgery, Tokyo Women's Medical University, Tokyo, Japan.
The accumulation of β2-microglobulin due to long-term hemodialysis is known as dialysis-related amyloidosis, a rare phenomenon that manifests as a subcutaneous mass. Subcutaneous β2-microglobulin amyloidomas are predominantly located on the buttocks. Owing to the load-bearing properties of this location and proximity to the anus, amyloidomas on the buttocks may be prone to pressure ulcers and infection.
View Article and Find Full Text PDFDialysis-related Amyloidosis Presenting as a Fever of Unknown Origin: Symptoms and Management.
Intern Med
December 2023
Department of Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, Japan.
A 74-year-old woman with a 34-year history of hemodialysis presented with an intermittent fever, which later coincided with recurrent bilateral shoulder and hip joint pain. Imaging studies suggested amyloid arthropathy, which was histologically confirmed by a synovial biopsy. Increasing β-microglobulin clearance during dialysis alone attenuated the intermittent fever and joint pain, but the symptoms did not disappear until the administration of prednisolone 10 mg/day.
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