Svetlana Lutsenko
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View article: Inactivation of Atp7b Copper Transporter in Intestinal Epithelial Cells Is Associated with Altered Lipid Processing and Cell Growth Machinery Independent from Hepatic Copper Accumulation and Severity of Liver Histology
Inactivation of Atp7b Copper Transporter in Intestinal Epithelial Cells Is Associated with Altered Lipid Processing and Cell Growth Machinery Independent from Hepatic Copper Accumulation and Severity of Liver Histology Open
The clinical manifestations of Wilson disease (WD) are related to copper accumulation in the liver and brain, but little is known about the role of other organs expressing the ATP7B copper transporter on metabolic and ultrastructural chang…
View article: Neuroprotection and metabolism: the role of adipokines in the pathogenesis and therapy of epilepsy
Neuroprotection and metabolism: the role of adipokines in the pathogenesis and therapy of epilepsy Open
Epilepsy remains one of the most widespread and socially significant chronic neurological diseases requiring the search for new therapeutic approaches. In recent years, more and more attention has been paid to the role of adipokines, biolo…
View article: Functional Screen of Wilson Disease ATP7B Variants Reveals Residual Transport Activities
Functional Screen of Wilson Disease ATP7B Variants Reveals Residual Transport Activities Open
Wilson disease is a disorder of copper (Cu) homeostasis caused by the malfunction of Cu transporter ATP7B and associated Cu accumulation in tissues. The existence of over 700 disease‐associated variants in the ATP7B gene and a broad spectr…
View article: Mammalian copper homeostasis: physiological roles and molecular mechanisms
Mammalian copper homeostasis: physiological roles and molecular mechanisms Open
In the past decade, evidence for the numerous roles of copper (Cu) in mammalian physiology has grown exponentially. The discoveries of Cu involvement in cell signaling, autophagy, cell motility, differentiation, and regulated cell death (c…
View article: Spatial Profiling of Metals through Matrix-Assisted Laser Desorption Ionization Mass Spectrometry Imaging
Spatial Profiling of Metals through Matrix-Assisted Laser Desorption Ionization Mass Spectrometry Imaging Open
The spatial-omic analysis of biomolecules such as nucleic acids, lipids, metabolites, and proteins is advancing the study of biological systems and processes in a physio-pathological context. Here, we describe an innovative matrix-assisted…
View article: Mechanism of Cu entry into the brain: many unanswered questions
Mechanism of Cu entry into the brain: many unanswered questions Open
Brain tissue requires high amounts of copper (Cu) for its key physiological processes, such as energy production, neurotransmitter synthesis, maturation of neuropeptides, myelination, synaptic plasticity, and radical scavenging. The requir…
View article: ATP7A-dependent copper sequestration contributes to termination of β-CATENIN signaling during early adipogenesis
ATP7A-dependent copper sequestration contributes to termination of β-CATENIN signaling during early adipogenesis Open
Cu buffering during early adipogenesis contributes to termination of β-catenin signaling. Abnormal upregulation of β-catenin was also observed in vivo in the livers of Atp7b-/- mice, which accumulate Cu, suggesting a tissue-independent cro…
View article: α-lipoic acid ameliorates consequences of copper overload by up-regulating selenoproteins and decreasing redox misbalance
α-lipoic acid ameliorates consequences of copper overload by up-regulating selenoproteins and decreasing redox misbalance Open
α-lipoic acid (LA) is an essential cofactor for mitochondrial dehydrogenases and is required for cell growth, metabolic fuel production, and antioxidant defense. In vitro, LA binds copper (Cu) with high affinity and as an endogenous membra…
View article: The role of the intestine in metabolic dysregulation in murine Wilson disease
The role of the intestine in metabolic dysregulation in murine Wilson disease Open
Background: The clinical manifestations of Wilson disease (WD) are related to copper accumulation in the liver and the brain, but little is known about other tissue involvement regarding metabolic changes in WD. In vitro studies suggested …
View article: P1B P-type ATPases: Cu<sup>+</sup>-ATPases in GtoPdb v.2023.1
P1B P-type ATPases: Cu<sup>+</sup>-ATPases in GtoPdb v.2023.1 Open
Copper-transporting ATPases convey copper ions across cell-surface and intracellular membranes. They consist of eight TM domains and associate with multiple copper chaperone proteins (e.g. ATOX1, O00244).
View article: SLC31 family of copper transporters in GtoPdb v.2023.1
SLC31 family of copper transporters in GtoPdb v.2023.1 Open
SLC31 family members, alongside the Cu-ATPases are involved in the regulation of cellular copper levels. The CTR1 transporter is a cell-surface transporter to allow monovalent copper accumulation into cells, while CTR2 appears to be a vacu…
View article: Supplementary Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma
Supplementary Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma Open
Supplementary Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma
View article: Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma
Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma Open
Purpose: Resistance to platinum chemotherapy remains a significant problem in ovarian carcinoma. Here, we examined the biological mechanisms and therapeutic potential of targeting a critical platinum resistance gene, ATP7B, using both in v…
View article: Supplementary Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma
Supplementary Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma Open
Supplementary Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma
View article: Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma
Data from Therapeutic Targeting of ATP7B in Ovarian Carcinoma Open
Purpose: Resistance to platinum chemotherapy remains a significant problem in ovarian carcinoma. Here, we examined the biological mechanisms and therapeutic potential of targeting a critical platinum resistance gene, ATP7B, using both in v…
View article: A Century of Progress on Wilson Disease and the Enduring Challenges of Genetics, Diagnosis, and Treatment
A Century of Progress on Wilson Disease and the Enduring Challenges of Genetics, Diagnosis, and Treatment Open
Wilson disease (WD) is a rare, inherited metabolic disorder manifested with varying clinical presentations including hepatic, neurological, psychiatric, and ophthalmological features, often in combination. Causative mutations in the ATP7B …
View article: The role of intestine in metabolic dysregulation in murine Wilson disease
The role of intestine in metabolic dysregulation in murine Wilson disease Open
Background and aims Major clinical manifestations of Wilson disease (WD) are related to copper accumulation in the liver and the brain, and little is known about other tissues involvement in metabolic changes in WD. In vitro studies sugges…
View article: Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain
Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain Open
Copper (Cu) has a multifaceted role in brain development, function, and metabolism. Two homologous Cu transporters, Atp7a (Menkes disease protein) and Atp7b (Wilson disease protein), maintain Cu homeostasis in the tissue. Atp7a mediates Cu…
View article: Heterogeneous nuclear ribonucleoprotein hnRNPA2/B1 regulates the abundance of the copper-transporter ATP7A in an isoform-dependent manner
Heterogeneous nuclear ribonucleoprotein hnRNPA2/B1 regulates the abundance of the copper-transporter ATP7A in an isoform-dependent manner Open
Copper (Cu) is an essential micronutrient with a critical role in mammalian growth and development. Imbalance of Cu causes severe diseases in humans; therefore, cellular Cu levels are tightly regulated. Major Cu-transport proteins and thei…
View article: Oxysterol misbalance critically contributes to Wilson disease pathogenesis
Oxysterol misbalance critically contributes to Wilson disease pathogenesis Open
Wilson disease (WD) is a metabolic disorder caused by inactivation of the copper-transporting ATPase 2 (ATP7B) and copper (Cu) overload in tissues. Excess Cu causes oxidative stress and pathologic changes with poorly understood mechanistic…
View article: Wilson Disease: Update on Pathophysiology and Treatment
Wilson Disease: Update on Pathophysiology and Treatment Open
Wilson disease (WD) is a potentially fatal genetic disorder with a broad spectrum of phenotypic presentations. Inactivation of the copper (Cu) transporter ATP7B and Cu overload in tissues, especially in the liver, are established causes of…
View article: Copper induces cell death by targeting lipoylated TCA cycle proteins
Copper induces cell death by targeting lipoylated TCA cycle proteins Open
Copper is an essential cofactor for all organisms, and yet it becomes toxic if concentrations exceed a threshold maintained by evolutionarily conserved homeostatic mechanisms. How excess copper induces cell death, however, is unknown. Here…
View article: Dynamic and cell-specific transport networks for intracellular copper ions
Dynamic and cell-specific transport networks for intracellular copper ions Open
Copper (Cu) homeostasis is essential for the development and function of many organisms. In humans, Cu misbalance causes serious pathologies and has been observed in a growing number of diseases. This Review focuses on mammalian Cu(I) tran…
View article: High Calorie Diet (Western Diet) in Atp7b-/- (Wilson Disease model) mice compared to C57BL/6J mice
High Calorie Diet (Western Diet) in Atp7b-/- (Wilson Disease model) mice compared to C57BL/6J mice Open
TMT proteomics Protein extracts were reduced with 10µL of 50mM ditheothreitol, alkylated with 10µL of 100mM iodoacetamide in the dark for 30 minutes and TCA/Acetone precipitated (100 µg). Protein pellets were resolubilized in 100µL of 100m…
View article: Nanobodies against the metal binding domains of ATP7B as tools to study copper transport in the cell
Nanobodies against the metal binding domains of ATP7B as tools to study copper transport in the cell Open
Nanobodies are genetically engineered single domain antibodies derived from the unusual heavy-chain only antibodies found in llamas and camels. The small size of the nanobodies and flexible selection schemes make them uniquely versatile to…