Loss of Homeostatic Microglia Signature in Prion Diseases
Standard
Loss of Homeostatic Microglia Signature in Prion Diseases. / Wang, Yue; Hartmann, Kristin; Thies, Edda; Mohammadi, Behnam; Altmeppen, Hermann; Sepulveda-Falla, Diego; Glatzel, Markus; Krasemann, Susanne.
in: CELLS-BASEL, Jahrgang 11, Nr. 19, 2948, 21.09.2022.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - Loss of Homeostatic Microglia Signature in Prion Diseases
AU - Wang, Yue
AU - Hartmann, Kristin
AU - Thies, Edda
AU - Mohammadi, Behnam
AU - Altmeppen, Hermann
AU - Sepulveda-Falla, Diego
AU - Glatzel, Markus
AU - Krasemann, Susanne
PY - 2022/9/21
Y1 - 2022/9/21
N2 - Prion diseases are neurodegenerative diseases that affect humans and animals. They are always fatal and, to date, no treatment exists. The hallmark of prion disease pathophysiology is the misfolding of an endogenous protein, the cellular prion protein (PrPC), into its disease-associated isoform PrPSc. Besides the aggregation and deposition of misfolded PrPSc, prion diseases are characterized by spongiform lesions and the activation of astrocytes and microglia. Microglia are the innate immune cells of the brain. Activated microglia and astrocytes represent a common pathological feature in neurodegenerative disorders. The role of activated microglia has already been studied in prion disease mouse models; however, it is still not fully clear how they contribute to disease progression. Moreover, the role of microglia in human prion diseases has not been thoroughly investigated thus far, and specific molecular pathways are still undetermined. Here, we review the current knowledge on the different roles of microglia in prion pathophysiology. We discuss microglia markers that are also dysregulated in other neurodegenerative diseases including microglia homeostasis markers. Data on murine and human brain tissues show that microglia are highly dysregulated in prion diseases. We highlight here that the loss of homeostatic markers may especially stand out.
AB - Prion diseases are neurodegenerative diseases that affect humans and animals. They are always fatal and, to date, no treatment exists. The hallmark of prion disease pathophysiology is the misfolding of an endogenous protein, the cellular prion protein (PrPC), into its disease-associated isoform PrPSc. Besides the aggregation and deposition of misfolded PrPSc, prion diseases are characterized by spongiform lesions and the activation of astrocytes and microglia. Microglia are the innate immune cells of the brain. Activated microglia and astrocytes represent a common pathological feature in neurodegenerative disorders. The role of activated microglia has already been studied in prion disease mouse models; however, it is still not fully clear how they contribute to disease progression. Moreover, the role of microglia in human prion diseases has not been thoroughly investigated thus far, and specific molecular pathways are still undetermined. Here, we review the current knowledge on the different roles of microglia in prion pathophysiology. We discuss microglia markers that are also dysregulated in other neurodegenerative diseases including microglia homeostasis markers. Data on murine and human brain tissues show that microglia are highly dysregulated in prion diseases. We highlight here that the loss of homeostatic markers may especially stand out.
KW - Animals
KW - Homeostasis
KW - Humans
KW - Mice
KW - Microglia/metabolism
KW - Neurodegenerative Diseases/metabolism
KW - Prion Diseases/metabolism
KW - Prion Proteins/metabolism
KW - Prions/metabolism
KW - Protein Isoforms/metabolism
U2 - 10.3390/cells11192948
DO - 10.3390/cells11192948
M3 - SCORING: Review article
C2 - 36230910
VL - 11
JO - CELLS-BASEL
JF - CELLS-BASEL
SN - 2073-4409
IS - 19
M1 - 2948
ER -