Loss of Homeostatic Microglia Signature in Prion Diseases

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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, Vol. 11, No. 19, 2948, 21.09.2022.

Research output: SCORING: Contribution to journalSCORING: Review articleResearch

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@article{ab8bd862ed3f44648dcc92de42136844,
title = "Loss of Homeostatic Microglia Signature in Prion Diseases",
abstract = "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.",
keywords = "Animals, Homeostasis, Humans, Mice, Microglia/metabolism, Neurodegenerative Diseases/metabolism, Prion Diseases/metabolism, Prion Proteins/metabolism, Prions/metabolism, Protein Isoforms/metabolism",
author = "Yue Wang and Kristin Hartmann and Edda Thies and Behnam Mohammadi and Hermann Altmeppen and Diego Sepulveda-Falla and Markus Glatzel and Susanne Krasemann",
year = "2022",
month = sep,
day = "21",
doi = "10.3390/cells11192948",
language = "English",
volume = "11",
journal = "CELLS-BASEL",
issn = "2073-4409",
publisher = "MDPI Multidisciplinary Digital Publishing Institute",
number = "19",

}

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 -