Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, often linked to mutations in the FUS gene, leading to toxic protein aggregates. This study investigates the role of HSP70, a molecular chaperone, in mitigating neurodegeneration in FUS-ALS mouse models. Using quantitativ...
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2024-12-01
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| author | Gennadii A. Piavchenko Ksenia S. Pokidova Egor A. Kuzmin Artem A. Venediktov Ilya Y. Izmailov Igor V. Meglinski Sergey L. Kuznetsov |
| author_facet | Gennadii A. Piavchenko Ksenia S. Pokidova Egor A. Kuzmin Artem A. Venediktov Ilya Y. Izmailov Igor V. Meglinski Sergey L. Kuznetsov |
| author_sort | Gennadii A. Piavchenko |
| collection | DOAJ |
| description | Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, often linked to mutations in the FUS gene, leading to toxic protein aggregates. This study investigates the role of HSP70, a molecular chaperone, in mitigating neurodegeneration in FUS-ALS mouse models. Using quantitative immunofluorescence microscopy, we mapped cellular changes in the primary motor cortex of double transgenic FUS/HSP70 mice and compared them to single FUS-transgenic controls. Our results reveal that double transgenic mice exhibit significantly reduced neuronal damage and increased levels of mature neuronal (NeuN) and microglial (Iba1) markers, indicating a protective effect of HSP70. Intracellular HSP70 expression proved more effective than extracellular release, suggesting that targeted HSP70 delivery to neurons may offer a promising therapeutic avenue for ALS. This study underscores the potential of quantitative immunofluorescence for mapping neuroprotective pathways and highlights HSP70’s impact on mitigating FUS-related pathology in ALS. |
| format | Article |
| id | doaj-art-46cc431f3e9c42c6a09d86aa5a100da6 |
| institution | Kabale University |
| issn | 2076-3417 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Applied Sciences |
| spelling | doaj-art-46cc431f3e9c42c6a09d86aa5a100da62024-12-27T14:07:50ZengMDPI AGApplied Sciences2076-34172024-12-0114241161410.3390/app142411614Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse ModelsGennadii A. Piavchenko0Ksenia S. Pokidova1Egor A. Kuzmin2Artem A. Venediktov3Ilya Y. Izmailov4Igor V. Meglinski5Sergey L. Kuznetsov6Department of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaDepartment of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaDepartment of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaDepartment of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaDepartment of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaDepartment of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaDepartment of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, RussiaAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, often linked to mutations in the FUS gene, leading to toxic protein aggregates. This study investigates the role of HSP70, a molecular chaperone, in mitigating neurodegeneration in FUS-ALS mouse models. Using quantitative immunofluorescence microscopy, we mapped cellular changes in the primary motor cortex of double transgenic FUS/HSP70 mice and compared them to single FUS-transgenic controls. Our results reveal that double transgenic mice exhibit significantly reduced neuronal damage and increased levels of mature neuronal (NeuN) and microglial (Iba1) markers, indicating a protective effect of HSP70. Intracellular HSP70 expression proved more effective than extracellular release, suggesting that targeted HSP70 delivery to neurons may offer a promising therapeutic avenue for ALS. This study underscores the potential of quantitative immunofluorescence for mapping neuroprotective pathways and highlights HSP70’s impact on mitigating FUS-related pathology in ALS.https://www.mdpi.com/2076-3417/14/24/11614heat shock proteinsHSP70HSPA1Aneurodegenerationprimary motor cortexamyotrophic lateral sclerosis |
| spellingShingle | Gennadii A. Piavchenko Ksenia S. Pokidova Egor A. Kuzmin Artem A. Venediktov Ilya Y. Izmailov Igor V. Meglinski Sergey L. Kuznetsov Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models Applied Sciences heat shock proteins HSP70 HSPA1A neurodegeneration primary motor cortex amyotrophic lateral sclerosis |
| title | Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models |
| title_full | Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models |
| title_fullStr | Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models |
| title_full_unstemmed | Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models |
| title_short | Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models |
| title_sort | quantitative immunofluorescence mapping of hsp70 s neuroprotective effects in fus als mouse models |
| topic | heat shock proteins HSP70 HSPA1A neurodegeneration primary motor cortex amyotrophic lateral sclerosis |
| url | https://www.mdpi.com/2076-3417/14/24/11614 |
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