Zobrazeno 1 - 10 of 20 pro vyhledávání: '"Maria B. Bagh"'
1
Akademický článek
Emerging new roles of the lysosome and neuronal ceroid lipofuscinoses
Autor: Anil B. Mukherjee, Abhilash P. Appu, Tamal Sadhukhan, Sydney Casey, Avisek Mondal, Zhongjian Zhang, Maria B. Bagh
Publikováno v: Molecular Neurodegeneration, Vol 14, Iss 1, Pp 1-23 (2019)
Abstract Neuronal Ceroid Lipofuscinoses (NCLs), commonly known as Batten disease, constitute a group of the most prevalent neurodegenerative lysosomal storage disorders (LSDs). Mutations in at least 13 different genes (called CLNs) cause various form
Externí odkaz: https://doaj.org/article/f4282e92961d482e86f406db5d20c556
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2
Akademický článek
Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model
Autor: Maria B. Bagh, Shiyong Peng, Goutam Chandra, Zhongjian Zhang, Satya P. Singh, Nagarajan Pattabiraman, Aiyi Liu, Anil B. Mukherjee
Publikováno v: Nature Communications, Vol 8, Iss 1, Pp 1-16 (2017)
Lysosomal acidification defects have been implicated in various neurodegenerative disorders. Baghet al. show that the V0a1 subunit of v-ATPase requires palmitoylation for correct sorting and trafficking to the lysosome membrane, and that such a proce
Externí odkaz: https://doaj.org/article/728ace11a95a4edda7a3da8550cb2ff6
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3
In a mouse model of INCL reduced S‐palmitoylation of cytosolic thioesterase APT1 contributes to microglia proliferation and neuroinflammation
Autor: Avisek Mondal, Abhilash P. Appu, Aiyi Liu, Wei Zhang, Tamal Sadhukhan, Maria B. Bagh, Anil B. Mukherjee
Publikováno v: Journal of Inherited Metabolic Disease. 44:1051-1069
S-palmitoylation is a reversible posttranslational modification in which a 16-carbon saturated fatty acid (generally palmitate) is attached to specific cysteine residues in polypeptides via thioester linkage. Dynamic S-palmitoylation (palmitoylation-
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::de7953dc0b205e7f66decc1e53ca92f6
https://doi.org/10.1002/jimd.12379
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4
Ppt1-deficiency dysregulates lysosomal Ca(++)-homeostasis contributing to pathogenesis in a mouse model of CLN1 disease
Autor: Avisek Mondal, Abhilash P. Appu, Tamal Sadhukhan, Maria B. Bagh, Rafael M. Previde, Sriparna Sadhukhan, Stanko Stojilkovic, Aiyi Liu, Anil B. Mukherjee
Publikováno v: J Inherit Metab Dis
Inactivating mutations in the PPT1 gene encoding palmitoyl-protein thioesterase-1 (PPT1) underlie the CLN1 disease, a devastating neurodegenerative lysosomal storage disorder. The mechanism of pathogenesis underlying CLN1 disease has remained elusive
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7c6fc0030ad8513538321c45bbc04e05
https://europepmc.org/articles/PMC9090967/
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5
Cln1 ‐mutations suppress Rab7‐RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis
Autor: Tamal Sadhukhan, Maria B. Bagh, Abhilash P. Appu, Chinmoy Sarkar, Arjun Saha, Goutam Chandra, Anil B. Mukherjee, Avisek Mondal
Publikováno v: J Inherit Metab Dis
Infantile neuronal ceroid lipofuscinosis (INCL) is a devastating neurodegenerative lysosomal storage disease (LSD) caused by inactivating mutations in the CLN1 gene. CLN1 encodes palmitoyl-protein thioesterase-1 (PPT1), a lysosomal enzyme that cataly
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::130aaf46740330c084b6d6cb0c6b7b13
https://doi.org/10.1002/jimd.12242
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6
Cln3‐mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl‐protein thioesterases‐1 (Ppt1)‐protein and Ppt1‐enzyme activity in the lysosome
Autor: Sydney Casey, Maria B. Bagh, Abhilash P. Appu, Tamal Sadhukhan, Avisek Mondal, Anil B. Mukherjee
Publikováno v: Journal of Inherited Metabolic Disease. 42:944-954
Mutations in at least 13 different genes (called CLNs) underlie various forms of neuronal ceroid lipofuscinoses (NCLs), a group of the most common neurodegenerative lysosomal storage diseases. While inactivating mutations in the CLN1 gene, encoding p
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::dda2bb58e6c08e55bb2b44a48c68ba2e
https://doi.org/10.1002/jimd.12106
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7
Ablation of microRNA-155 and neuroinflammation in a mouse model of CLN1-disease
Autor: Sriparna Sadhukhan, Maria B. Bagh, Abhilash P. Appu, Tamal Sadhukhan, Tianwei Li, Steven L. Coon, Avisek Mondal, James R. Iiben, Anil B. Mukherjee
Publikováno v: Biochemical and biophysical research communications. 571
Infantile neuronal ceroid lipofuscinosis (INCL), also known as CLN1-disease, is a devastating neurodegenerative lysosomal storage disorder (LSD), caused by inactivating mutations in the CLN1 gene. The Cln1−/− mice, which mimic INCL, manifest prog
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1c563d61ee89f4e7f68e1aaf45beac84
https://pubmed.ncbi.nlm.nih.gov/34325129
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10
Emerging new roles of the lysosome and neuronal ceroid lipofuscinoses
Autor: Zhongjian Zhang, Sydney Casey, Tamal Sadhukhan, Maria B. Bagh, Anil B. Mukherjee, Abhilash P. Appu, Avisek Mondal
Publikováno v: Molecular Neurodegeneration
Molecular Neurodegeneration, Vol 14, Iss 1, Pp 1-23 (2019)
Neuronal Ceroid Lipofuscinoses (NCLs), commonly known as Batten disease, constitute a group of the most prevalent neurodegenerative lysosomal storage disorders (LSDs). Mutations in at least 13 different genes (called CLNs) cause various forms of NCLs
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::aff5d00ea825671906dd096a06f177a9
https://pubmed.ncbi.nlm.nih.gov/30651094
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