Results 201 to 210 of about 37,618 (246)
The FEBS Journal, EarlyView.Exercise‐related microRNAs cel‐miR‐249‐3p and cel‐miR‐77‐5p in C. elegans regulate lifespan, fitness, mitochondrial morphology and membrane potential. Although lacking direct mammalian orthologs, H2O2‐induced microRNAs mmu‐miR‐181a‐5p and mmu‐miR‐378a‐3p regulate myogenesis, autophagy, mitochondrial content and respiration in murine myoblasts ...
Qin Xia +7 more
wiley +1 more source
The FEBS Journal, EarlyView.Divalent metal transporter 1 (Dmt1) maintains iron homeostasis and lysosomal proteostasis required for physiological Notch receptor–ligand signaling. Dmt1 loss lowers iron storage capacity (ferritin), increasing intracellular Fe2+, driving ROS and lipid peroxidation, and leading to lysosomal/mitochondrial dysfunction.
Rui Zhang +5 more
wiley +1 more source
Spatially resolved mapping of histones reveals selective neuronal response in Rett syndrome
The FEBS Journal, EarlyView.Loss of Mecp2 function is associated with Rett syndrome (RTT). MeCP2 regulates chromatin, yet its influence on histone composition and dynamics is unclear. Combining MALDI‐MSI with LCM–LC–MS/MS, we mapped histone proteoforms across the dentate gyrus, cornu ammonis, and cerebellum in two mouse models of RTT.
Frederike Schäfer +6 more
wiley +1 more source
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Lysosomal storage disorders (LSDs) are a group of inherited metabolic diseases caused by dysfunction of the lysosomal system, with subsequent progressive accumulation of macromolecules, activation of inflammatory response, and cell death. Neurologic damage is almost always present, and it is usually degenerative.
Alessandro P, Burlina +2 more
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Lysosomal storage disorders (LSDs) are a group of inherited metabolic diseases caused by dysfunction of the lysosomal system, with subsequent progressive accumulation of macromolecules, activation of inflammatory response, and cell death. Neurologic damage is almost always present, and it is usually degenerative.
Alessandro P, Burlina +2 more
+6 more sources
Neuropathology and Applied Neurobiology, 1978
The majority of lysosomal storage diseases affect the central nervous system. Those that reflect a primary lysosomal disorder are associated with genetically determined deficiencies of specific lysosomal enzymes and storage of the relevant substrate. Autofluorescent lipopigments accumulate in the ceroid‐lipofuscinoses, a heterogeneous group of diseases
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The majority of lysosomal storage diseases affect the central nervous system. Those that reflect a primary lysosomal disorder are associated with genetically determined deficiencies of specific lysosomal enzymes and storage of the relevant substrate. Autofluorescent lipopigments accumulate in the ceroid‐lipofuscinoses, a heterogeneous group of diseases
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Lysosomal Glycosphingolipid Storage Diseases
Annual Review of Biochemistry, 2019Glycosphingolipids are cell-type-specific components of the outer leaflet of mammalian plasma membranes. Gangliosides, sialic acid–containing glycosphingolipids, are especially enriched on neuronal surfaces. As amphi-philic molecules, they comprise a hydrophilic oligosaccharide chain attached to a hydrophobic membrane anchor, ceramide.
Bernadette, Breiden, Konrad, Sandhoff
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Current Treatment Options in Neurology, 2001
Lysosomal storage disorders (LSDs), over 40 different diseases, are now considered treatable disorders. Only a few short years ago, Lysosomal storage disorders were seen as interesting neurodegenerative disorders without any potential for treatment. Effective treatment strategies such as bone marrow transplantation (BMT), enzyme replacement therapy ...
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Lysosomal storage disorders (LSDs), over 40 different diseases, are now considered treatable disorders. Only a few short years ago, Lysosomal storage disorders were seen as interesting neurodegenerative disorders without any potential for treatment. Effective treatment strategies such as bone marrow transplantation (BMT), enzyme replacement therapy ...
openaire +2 more sources
Storage problems in lysosomal diseases
Biochemical Society Transactions, 2010Biochemical disorders in lysosomal storage diseases consist of the interruption of metabolic pathways involved in the recycling of the degradation products of one or several types of macromolecules. The progressive accumulation of these primary storage products is the direct consequence of the genetic defect and represents the initial pathogenic event.
Jean Michel, Heard +5 more
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Lysosomal Storage Diseases in Adults
Pathology - Research and Practice, 1994Most lysosomal storage disorders are known as pediatric diseases. In recent years late onset and adult forms of these disorders have been recognized. The adult form of a given lysosomal storage disorder differs from the childhood disease in several respects. Adult disorders are, with some exceptions, less common than the childhood diseases.
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