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View article: Efficacy and safety of efavirenz in Niemann-Pick disease type C
Efficacy and safety of efavirenz in Niemann-Pick disease type C Open
View article: Sphingomyelin-induced glucocorticoid receptor alterations lead to impaired presynaptic plasticity in acid sphingomyelinase deficient neurons
Sphingomyelin-induced glucocorticoid receptor alterations lead to impaired presynaptic plasticity in acid sphingomyelinase deficient neurons Open
View article: Secondary accumulation of lyso-platelet activating factors in lysosomal storage diseases
Secondary accumulation of lyso-platelet activating factors in lysosomal storage diseases Open
Lysosomal storage diseases (LSDs) are a group of inherited disorders caused by defects in genes that encode lysosomal enzymes, transmembrane proteins, or transport proteins. These defects typically lead to the accumulation of undegraded su…
View article: Sex-dependent upregulation in oxylipins involved in inflammation resolution in the cerebellum of Niemann-Pick disease C1 mice
Sex-dependent upregulation in oxylipins involved in inflammation resolution in the cerebellum of Niemann-Pick disease C1 mice Open
Unresolved inflammation in the cerebellum is implicated in motor and cognitive decline in Niemann-Pick disease type C (NPC), a neurodegenerative lysosomal storage disorder caused by pathogenic mutations in the Npc1 gene encoding a choleste…
View article: Enhanced mGluR5 intracellular activity causes psychiatric alterations in Niemann Pick type C disease
Enhanced mGluR5 intracellular activity causes psychiatric alterations in Niemann Pick type C disease Open
View article: Neurons regulate the esterification of bioactive lipid mediators in the brain of acid sphingomyelinase deficient mice
Neurons regulate the esterification of bioactive lipid mediators in the brain of acid sphingomyelinase deficient mice Open
View article: TRANSCRIPTOMIC ANALYSIS OF ACID SPHINGOMYELINASE KNOCKOUT OLIGODENDROCYTES: UNCOVERING KEY MOLECULAR PATHWAYS AND PROCESSES IN ASM-ASSOCIATED DISEASES
TRANSCRIPTOMIC ANALYSIS OF ACID SPHINGOMYELINASE KNOCKOUT OLIGODENDROCYTES: UNCOVERING KEY MOLECULAR PATHWAYS AND PROCESSES IN ASM-ASSOCIATED DISEASES Open
View article: CHARACTERIZATION AND RESCUE OF THE ALTERATIONS IN OLIGODENDROCYTES MODELING THE ACID SPHINGOMYELINASE DEFICIENCY
CHARACTERIZATION AND RESCUE OF THE ALTERATIONS IN OLIGODENDROCYTES MODELING THE ACID SPHINGOMYELINASE DEFICIENCY Open
View article: NASAL DELIVERY OF GLUCOCORTICOIDS ENCAPSULATED IN CHITOSAN NANOPARTICLES FOR THE TREATMENT OF BRAIN PATHOLOGY IN THE ACID SPHINGOMYELINASE DEFICIENCY.
NASAL DELIVERY OF GLUCOCORTICOIDS ENCAPSULATED IN CHITOSAN NANOPARTICLES FOR THE TREATMENT OF BRAIN PATHOLOGY IN THE ACID SPHINGOMYELINASE DEFICIENCY. Open
View article: Ellagic acid and its metabolites urolithins A/B ameliorate most common disease phenotypes in cellular and mouse models for lysosomal storage disorders by enhancing extracellular vesicle secretion
Ellagic acid and its metabolites urolithins A/B ameliorate most common disease phenotypes in cellular and mouse models for lysosomal storage disorders by enhancing extracellular vesicle secretion Open
View article: “MiR-7 controls cholesterol biosynthesis through posttranscriptional regulation of DHCR24 expression”
“MiR-7 controls cholesterol biosynthesis through posttranscriptional regulation of DHCR24 expression” Open
View article: Sphingomyelin 16:0 is a therapeutic target for neuronal death in acid sphingomyelinase deficiency
Sphingomyelin 16:0 is a therapeutic target for neuronal death in acid sphingomyelinase deficiency Open
Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder caused by mutations in the SMPD1 gene encoding for the acid sphingomyelinase (ASM). While intravenous infusion of recombinant ASM is an effective treatment for the per…
View article: Efficacy and safety clinical trial with efavirenz in patients diagnosed with adult Niemann-pick type C with cognitive impairment
Efficacy and safety clinical trial with efavirenz in patients diagnosed with adult Niemann-pick type C with cognitive impairment Open
Background: Niemann-Pick disease Type C (NPC) is a genetic, incurable, neurodegenerative disorder. This orphan disease is most frequently caused by mutations in the NPC1 protein, resulting in intralysossomal cholesterol accumulation. NPC1 …
View article: Models to study basic and applied aspects of lysosomal storage disorders
Models to study basic and applied aspects of lysosomal storage disorders Open
The lack of available treatments and fatal outcome in most lysosomal storage disorders (LSDs) have spurred research on pathological mechanisms and novel therapies in recent years. In this effort, experimental methodology in cellular and an…
View article: New paradigms for the treatment of lysosomal storage diseases: targeting the endocannabinoid system as a therapeutic strategy
New paradigms for the treatment of lysosomal storage diseases: targeting the endocannabinoid system as a therapeutic strategy Open
View article: CERTL reduces C16 ceramide, amyloid-β levels and inflammation in a model of Alzheimer's disease
CERTL reduces C16 ceramide, amyloid-β levels and inflammation in a model of Alzheimer's disease Open
Background: Dysregulation of ceramide and sphingomyelin levels have been suggested to contribute to the pathogenesis of Alzheimer’s disease (AD). Ceramide transfer proteins (CERTs) are ceramide carriers which are crucial for ceramide and s…
View article: CERTL reduces C16 ceramide, amyloid-β levels and inflammation in a model of Alzheimer's disease
CERTL reduces C16 ceramide, amyloid-β levels and inflammation in a model of Alzheimer's disease Open
Background: Dysregulation of ceramide and sphingomyelin levels have been suggested to contribute to the pathogenesis of Alzheimer’s disease (AD). Ceramide transfer proteins (CERTs) are ceramide carriers, crucial for ceramide and sphingomye…
View article: Editorial: Lipids in the Brain
Editorial: Lipids in the Brain Open
aimed at studying them as biologically active molecules essential for cell fate, especially in the central nervous system (CNS).This special issue includes nine papers, five reviews and four research articles, covering some of the more rec…
View article: CERTL Reduces C16 Ceramide, Amyloid-β Levels and Inflammation in a Model of Alzheimer's Disease
CERTL Reduces C16 Ceramide, Amyloid-β Levels and Inflammation in a Model of Alzheimer's Disease Open
Background: Deregulation of ceramide and sphingomyelin levels have been suggested to contribute to the pathogenesis of Alzheimer’s disease (AD). Ceramide transfer proteins (CERTs) are ceramide carriers, crucial for ceramide and sphingomyel…
View article: CERTL Reduces C16 Ceramide, Amyloid-β Levels and Inflammation in a Model of Alzheimer's Disease
CERTL Reduces C16 Ceramide, Amyloid-β Levels and Inflammation in a Model of Alzheimer's Disease Open
Background: Deregulation of ceramide and sphingomyelinlevels have been suggested tocontribute tothe pathogenesis of Alzheimer’s disease (AD).Ceramide transfer proteins (CERTs) are ceramide carriers, crucial for ceramide and sphingomyelin b…
View article: Brain, Mind and Behaviour
Brain, Mind and Behaviour Open
The study of the brain will tell us what makes us humans and how our social behavior generates. Increasing our understanding of how the brain functions and interactswith the ecosystem to interpretthe world will not only help to find effect…
View article: <scp>NPC</scp> 1 enables cholesterol mobilization during long‐term potentiation that can be restored in Niemann–Pick disease type C by <scp>CYP</scp> 46A1 activation
<span>NPC</span> 1 enables cholesterol mobilization during long‐term potentiation that can be restored in Niemann–Pick disease type C by <span>CYP</span> 46A1 activation Open
View article: Adeno-associated viral vector serotype 9–based gene therapy for Niemann-Pick disease type A
Adeno-associated viral vector serotype 9–based gene therapy for Niemann-Pick disease type A Open
Gene therapy for Niemann-Pick type A disease is safe in nonhuman primates and has therapeutic effects in a mouse model.
View article: SGPL1 (sphingosine phosphate lyase 1) modulates neuronal autophagy via phosphatidylethanolamine production
SGPL1 (sphingosine phosphate lyase 1) modulates neuronal autophagy via phosphatidylethanolamine production Open
Macroautophagy/autophagy defects have been identified as critical factors underlying the pathogenesis of neurodegenerative diseases. The roles of the bioactive signaling lipid sphingosine-1-phosphate (S1P) and its catabolic enzyme SGPL1/SP…
View article: Lysosomal cell death mechanisms in aging
Lysosomal cell death mechanisms in aging Open
View article: Host sphingomyelin increases West Nile virus infection in vivo
Host sphingomyelin increases West Nile virus infection in vivo Open
View article: PrP<sup>C</sup> co-immunolabeling with synaptophysin (left) and PSD95 (right).
PrP<sup>C</sup> co-immunolabeling with synaptophysin (left) and PSD95 (right). Open
Confocal images of fully developed hippocampal primary neurons (21 DIV). Normal (above) and surface immunolabeling (below) of PrPC (green) coupled with synaptophysin and PSD95 (red). Antibodies used: D18 (10 µg/mL and 20 µg/mL i…
View article: Mitochondrial Respiration Controls Lysosomal Function during Inflammatory T Cell Responses
Mitochondrial Respiration Controls Lysosomal Function during Inflammatory T Cell Responses Open