Publisher Correction: Altered perivascular fibroblast activity precedes ALS disease onset

Månberg, Anna; Skene, Nathan; Sanders, Folkert; Trusohamn, Marta; Remnestål, Julia; Szczepińska, Anna; Aksoylu, Inci Sevval; Lönnerberg, Peter; Ebarasi, Lwaki; Wouters, Stefan; Lehmann, Manuela; Olofsson, Jennie; Antequera, Inti von Gohren; Domaniku, Aylin; Schaepdryver, Maxim De; Vocht, Joke De; Poesen, Koen; Uhlén, Mathias; Anink, Jasper J.; Mijnsbergen, Caroline; Vergunst-Bosch, Hermieneke; Hübers, Annemarie; Kläppe, Ulf; Rodriguez‐Vieitez, Elena; Gilthorpe, Jonathan; Hedlund, Eva; Harris, R. Adron; Aronica, Eleonora; Damme, Philip Van; Ludolph, Albert C.; Veldink, Jan H.; Ingre, Caroline; Nilsson, Peter M.; Lewandowski, Sebastian

doi:10.1038/s41591-021-01414-6

Cited by 6 publications

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“…In addition, fibroblasts transmit signals through the bloodstream, affecting the progression of neurodegenerative diseases. ALS patients in whom fibroblast protein product accumulates in perivascular space have shorter survival times [95]. In fact, as one of the indispensable components of the vasculature, the function of fibroblasts is somewhat unclear, and there may be unknown functions in SMA [85].…”

Section: Discussionmentioning

confidence: 99%

Single-cell RNA sequencing reveals dysregulation of spinal cord cell types in a severe spinal muscular atrophy mouse model

Sun

Qiu

Yang

et al. 2022

Preprint

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Although spinal muscular atrophy (SMA) is a motor neuron disease caused by the loss of survival of motor neuron proteins, there is growing evidence that lesions in nonmotor neuron cells are involved in SMA pathogenesis. Transcriptome alterations occurring at the single-cell level in SMA spinal cord are unknown. Here, we performed single-cell RNA sequencing of the spinal cord of a severe SMA mouse model, and characterized ten cell types as well as their differentially expressed genes. Using CellChat, we found that cellular communication between different cell types in the spinal cord of SMA mice is substantially reduced. A dimensionality reduction analysis revealed 29 cell subtypes and differentially expressed genes for each subtype. Among them, the most significant change in the SMA spinal cord at the single-cell level was observed in a subpopulation of vascular fibroblasts. This subpopulation was greatly reduced, which may be responsible for vascular defects in SMA mice and may lead to widespread reductions in protein synthesis and energy metabolism. This study is the first to report a single-cell atlas of the spinal cord of mice with severe SMA, providing new insights into SMA pathogenesis.

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Section: Discussionmentioning

confidence: 99%

Single-cell RNA sequencing reveals dysregulation of spinal cord cell types in a severe spinal muscular atrophy mouse model

Sun

Qiu

Yang

et al. 2022

Preprint

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“…Astrocytes are hypothesized as significant actors in ALS and FTD progression mainly due to results from in vitro and in vivo rodent models ( Izrael et al, 2020 ). Analyses of human post-mortem spinal cord tissue revealed enrichment of astrocyte-specific genes and enlarged perivascular spaces with separation of astrocyte and mural basement membranes in sALS ( Månberg et al, 2021 ). RNA-seq datasets implicated enrichment of upregulated DEGs related to astrocyte functions in ALS compared to control spinal cord samples ( Wang et al, 2021 ).…”

Section: Breaking Bad: When Do Astrocytes Become Toxic To Surrounding...mentioning

confidence: 99%

The Multifaceted Neurotoxicity of Astrocytes in Ageing and Age-Related Neurodegenerative Diseases: A Translational Perspective

et al. 2022

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In a healthy physiological context, astrocytes are multitasking cells contributing to central nervous system (CNS) homeostasis, defense, and immunity. In cell culture or rodent models of age-related neurodegenerative diseases (NDDs), such as Alzheimer’s disease (AD) and Parkinson’s disease (PD), numerous studies have shown that astrocytes can adopt neurotoxic phenotypes that could enhance disease progression. Chronic inflammatory responses, oxidative stress, unbalanced phagocytosis, or alteration of their core physiological roles are the main manifestations of their detrimental states. However, if astrocytes are directly involved in brain deterioration by exerting neurotoxic functions in patients with NDDs is still controversial. The large spectrum of NDDs, with often overlapping pathologies, and the technical challenges associated with the study of human brain samples complexify the analysis of astrocyte involvement in specific neurodegenerative cascades. With this review, we aim to provide a translational overview about the multi-facets of astrocyte neurotoxicity ranging from in vitro findings over mouse and human cell-based studies to rodent NDDs research and finally evidence from patient-related research. We also discuss the role of ageing in astrocytes encompassing changes in physiology and response to pathologic stimuli and how this may prime detrimental responses in NDDs. To conclude, we discuss how potentially therapeutic strategies could be adopted to alleviate or reverse astrocytic toxicity and their potential to impact neurodegeneration and dementia progression in patients.

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Integrative transcriptomic analysis of the amyotrophic lateral sclerosis spinal cord implicates glial activation and suggests new risk genes

et al. 2022

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Publisher Correction: Altered perivascular fibroblast activity precedes ALS disease onset

Abstract: In the version of this article initially published, the label along the right margin of the top row in Fig. 2d (SO1D G93A ) was incorrect. The correct label is 'SOD1 G93A ' . The error has been corrected in the HTML and PDF versions of the article.

Cited by 6 publications

References 0 publications

Single-cell RNA sequencing reveals dysregulation of spinal cord cell types in a severe spinal muscular atrophy mouse model

Single-cell RNA sequencing reveals dysregulation of spinal cord cell types in a severe spinal muscular atrophy mouse model

The Multifaceted Neurotoxicity of Astrocytes in Ageing and Age-Related Neurodegenerative Diseases: A Translational Perspective

Integrative transcriptomic analysis of the amyotrophic lateral sclerosis spinal cord implicates glial activation and suggests new risk genes

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