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Zebra Bodies in the Kidney: Is it a Pathognomonic Finding of Fabry Disease?
Indian J Nephrol
June 2024
Department of Microbiology, All India Institute of Medical Sciences, Patna, Bihar, India.
Zebra bodies are intralysosomal lamellar inclusion bodies. It is accepted as a specific feature of Fabry disease. However, it has been reported in many hereditary and acquired conditions.
View Article and Find Full Text PDFZebra Bodies in Kidney Biopsy: Drug-Induced Phospholipidosis in a Patient With Systemic Lupus Erythematosus.
J Rheumatol
March 2025
Chris T. Derk, MD, MS, Clinical Medicine, Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
The importance of a multidisciplinary approach in two tricky cases: the perfect match for Fabry disease.
BMC Nephrol
February 2025
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum - University of Bologna, Bologna, Italy.
Anderson-Fabry disease (AFD) is a multisystem X-linked lysosomal storage disorder caused by a deficiency in the enzyme α-galactosidase A (α-Gal A). This deficiency results in the intracellular accumulation of glycosphingolipids, primarily uncleaved globotriaosylceramide (Gb3) and its deacylated form, lyso-globotriaosylceramide (Lyso-Gb3), leading to progressive organ damage and functional impairment. The diagnostic evaluation for AFD involves clinical assessment and family history, supported by biochemical testing (α-Gal A enzyme activity and Lyso-Gb3 levels) and genetic analysis of the GLA gene.
View Article and Find Full Text PDFα-Synuclein orchestrates Th17 responses as antigen and adjuvant in Parkinson's disease.
J Neuroinflammation
February 2025
Department of Immunology, Juntendo University, Faculty of Medicine, Bunkyo-ku, Tokyo, Japan.
Recently, the role of T cells in the pathology of α-synuclein (αS)-mediated neurodegenerative disorders called synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy, has attracted increasing attention. Although the existence of αS-specific T cells and the immunogenicity of the post-translationally modified αS fragment have been reported in PD and DLB, the key cellular subset associated with disease progression and its induction mechanism remain largely unknown.Peripheral blood mononuclear cells (PBMCs) from synucleinopathy patients and healthy controls were cultured in the presence of the αS peptide pools.
View Article and Find Full Text PDFThe effect of dietary camelina, flaxseed, and canola oil supplementation on skin fatty acid profile and immune and inflammatory responses in healthy adult horses.
J Anim Sci
January 2025
Department of Animal Biosciences, University of Guelph, Guelph, ON, Canada N1G 2W1.
Camelina sativa is an oilseed crop rich in α-linolenic acid (ALA), an n-3 fatty acid (FA), and is resistant to harsh climates and pests. Previously, supplementation with camelina oil (CAM) in horses had no adverse effects on basic health parameters and had comparable skin and coat parameters as both flaxseed oil (FLX) and canola oil (OLA). Further, the plasma FA profile of horses was reflective of their respective treatment oil.
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