Cited by 110Protein Kinase Regulation and GTPase SignalingAlzheimer's disease research and treatmentsNeuroscience and Neuropharmacology Research

The DOI matches its Crossref record; 231 of the 233 references it cites were found in a registry or an index; 165 of the 248 sources its claims cite were checked for support.

RhoA Signaling in Neurodegenerative Diseases

Open access

Abstract

Ras homolog gene family member A (RhoA) is a small GTPase of the Rho family involved in regulating multiple signal transduction pathways that influence a diverse range of cellular functions. RhoA and many of its downstream effector proteins are highly expressed in the nervous system, implying an important role for RhoA signaling in neurons and glial cells. Indeed, emerging evidence points toward a role of aberrant RhoA signaling in neurodegenerative diseases such as Parkinson’s disease, Alzheimer’s disease, Huntington’s disease, and amyotrophic lateral sclerosis. In this review, we summarize the current knowledge of RhoA regulation and downstream cellular functions with an emphasis on the role of RhoA signaling in neurodegenerative diseases and the therapeutic potential of RhoA inhibition in neurodegeneration.

Overview

AI snapshot

Written from the paper’s full text.

TL;DR

This review summarizes RhoA regulation and downstream functions, emphasizing aberrant RhoA/ROCK signaling in Parkinson’s disease, Alzheimer’s disease, Huntington’s disease, and amyotrophic lateral sclerosis. It concludes that inhibiting RhoA signaling has disease-modifying potential in preclinical models but requires further mechanistic investigation.

Key findings

  • RhoA is one of 20 human Rho GTPase family members and is controlled by GDP/GTP cycling, GEFs, GAPs, GDIs, GDFs, post-translational modifications including prenylation at Cys190, and miRNAs of 17–24 nucleotides; only three RhoGDIs have been described in mammals.
  • RhoA/ROCK signaling regulates cytoskeletal dynamics, cell death, mitochondrial fission/mitophagy, autophagy, neuroinflammation, and gene transcription, with many RhoA effectors highly expressed in the nervous system.
  • In disease models, increased RhoA/ROCK signaling is implicated in PD, AD, HD, and ALS; RhoA/ROCK inhibition protects dopaminergic neurons, reduces α-synuclein/tau/Aβ/Htt pathology, improves motor and cognitive outcomes, and extends survival in SOD1G93A mice.
  • A high-throughput screen of ~3000 compounds identified ROCK inhibitors that increased Parkin recruitment to damaged mitochondria, with SR3677 being the most efficient and neuroprotective in a fly PD model.

Methods

  • Narrative review; no systematic review protocol, search dates, databases, or inclusion/exclusion criteria are stated.
  • Synthesizes published literature on RhoA structure/regulation, downstream targets, and studies in PD, AD, HD, and ALS, including human postmortem tissue, patient blood, iPSC-derived neurons, primary neuronal cultures, cell lines, and rodent/Drosophila models.
  • Main analysis is qualitative synthesis; Table 3 summarizes the studies discussed and their RhoA inhibitory strategies.

Limitations

  • Stated: the exact downstream targets of RhoA/ROCK responsible for autophagy effects are not fully elucidated, and more studies are needed; mechanisms underlying the disease-modifying potential in HD need further investigation; future research should investigate aberrant downstream pathways.
  • Stated: some ALS studies applied ROCK inhibition at the presymptomatic stage, and a more advanced-stage SOD1G93A study did not show increased motor neuron survival or reduced microglial infiltration, although motor function improved in male mice.
  • Evident: as a narrative review, it lacks systematic search and quality assessment and reports no primary data or effect sizes, so selection bias is possible.

Model-written summary. Check it against the paper before relying on it.

Integrity signals

Registry

Crossref · 10.3390/cells11091520

Cites

231 of 231 references verified0 citation errors · 0 not checkable

Statistics

Nothing to recompute.

Figures

Figure 1 RhoA protein domains and 3D structure. (A) The RHOA gene is located on the short arm of chromosome 3 (3p21.3). (B) RhoA is a monomeric protein comprised of 193 amino acids and a molecular mass of 21.7 kDa. The different domains and their amino acid positions are marked. The G-domain responsible for nucleotide binding contains five motifs, G1–G5, where the G2 and G3 motifs are located in the switch I and II regions that change conformation in response to GTP/GDP binding. The insert domain and the hypervariable C-terminus are involved in regulatory or effector protein binding, while the CAAX motif in the C-terminal end undergoes post-translational lipid modification crucial for membrane targeting. (C) 3D structure and conformation of the RhoA–GDP complex and the RhoA–GTP complex. Marked in blue is the P-loop (G1 motif) that binds the β,γ-phosphate of the guanine nucleotide and a Mg2+ ion. Marked in red are the switch I and II regions responsible for the conformational change of the protein in response to GTP/GDP binding.
Figure 1 RhoA protein domains and 3D structure. (A) The RHOA gene is located on the short arm of chromosome 3 (3p21.3). (B) RhoA is a monomeric protein comprised of 193 amino acids and a molecular mass of 21.7 kDa. The different domains and their amino acid positions are marked. The G-domain responsible for nucleotide binding contains five motifs, G1–G5, where the G2 and G3 motifs are located in the switch I and II regions that change conformation in response to GTP/GDP binding. The insert domain and the hypervariable C-terminus are involved in regulatory or effector protein binding, while the CAAX motif in the C-terminal end undergoes post-translational lipid modification crucial for membrane targeting. (C) 3D structure and conformation of the RhoA–GDP complex and the RhoA–GTP complex. Marked in blue is the P-loop (G1 motif) that binds the β,γ-phosphate of the guanine nucleotide and a Mg2+ ion. Marked in red are the switch I and II regions responsible for the conformational change of the protein in response to GTP/GDP binding.CC BY · PubMed Central
Figure 2 RhoA regulation. (A) RhoA cycle between an active GTP-bound conformation at the plasma membrane and an inactive GDP-bound conformation in the cytosol facilitated by prenylation and different regulatory proteins: guanine nucleotide exchange factors (GEFs), GTPase-activating proteins (GAPs), guanine nucleotide dissociation inhibitors (GDIs), and GDI displacement factors (GDFs).(B) Post-translational modification events mapped onto the amino acid sequence of RhoA with highlighted locations of the functional protein domains from Figure 1. (C) Transcriptional and post-transcriptional regulation of RhoA showing known transcription factors and miRNAs regulating RHOA transcription.
Figure 2 RhoA regulation. (A) RhoA cycle between an active GTP-bound conformation at the plasma membrane and an inactive GDP-bound conformation in the cytosol facilitated by prenylation and different regulatory proteins: guanine nucleotide exchange factors (GEFs), GTPase-activating proteins (GAPs), guanine nucleotide dissociation inhibitors (GDIs), and GDI displacement factors (GDFs).(B) Post-translational modification events mapped onto the amino acid sequence of RhoA with highlighted locations of the functional protein domains from Figure 1. (C) Transcriptional and post-transcriptional regulation of RhoA showing known transcription factors and miRNAs regulating RHOA transcription.CC BY · PubMed Central
Figure 3 RhoA signaling. RhoA transmits signals from a variety of different cell surface receptors, such as integrins, tyrosine kinase receptors, G protein-coupled receptors, and cytokine receptors, by interacting with downstream effector molecules involved in many cellular processes. These include, Golgi function, gene transcription, inflammation, autophagy, cell death, cytoskeletal modulation, mitochondrial homeostasis, and cytokinesis.
Figure 3 RhoA signaling. RhoA transmits signals from a variety of different cell surface receptors, such as integrins, tyrosine kinase receptors, G protein-coupled receptors, and cytokine receptors, by interacting with downstream effector molecules involved in many cellular processes. These include, Golgi function, gene transcription, inflammation, autophagy, cell death, cytoskeletal modulation, mitochondrial homeostasis, and cytokinesis.CC BY · PubMed Central

Verification

Claims the paper makes

66 Supported · 53 Partly supported · 45 No full text of the sources · 12 Own interpretation · 8 Not checked yet · 3 Supported so far · 3 Not supported · 3 Couldn’t confirm · 1 Mixed support · 1 No basis given · accuracy not yet measured

Show all 100 claims

  • Probably not a substantial claim. 29
  • It may state two separate claims as one. 73
95 of 95
  • The paper relies on this without support.

    Findings from cellular and animal models of neurodegenerative diseases predict therapeutic effects of RhoA/ROCK inhibition in human patients.

    “These data suggest that RhoA/ROCK inhibition might be a potential therapeutic strategy for PD as it interferes with both neuritic and axonal phenotypes, mitochondrial and autophagic impairments, α-synuclein pathology, and neuroinflammation.”
    “In view of these data, the inhibition of RhoA signaling to combat Aβ and tau-mediated pathologies in AD holds great potential.”
    “These data demonstrate that inhibition of RhoA signaling has a disease-modifying potential in HD.”
  • The paper relies on this without support.

    RhoA signaling is involved in cytoskeleton modulation and cell death.

    “This review focused on the specific role of RhoA signaling as this is involved in cytoskeleton modulation and cell death and has recently been linked to neurodegeneration-relevant cellular processes such as mitochondrial homeostasis, autophagy, and neuroinflammation.”
  • The paper relies on this without support.

    RhoA signaling has recently been linked to neurodegeneration-relevant cellular processes such as mitochondrial homeostasis, autophagy, and neuroinflammation.

    “This review focused on the specific role of RhoA signaling as this is involved in cytoskeleton modulation and cell death and has recently been linked to neurodegeneration-relevant cellular processes such as mitochondrial homeostasis, autophagy, and neuroinflammation.”
3 of 3

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Metadata

Authors
Schmidt, S. I., Blaabjerg, M., Freude, K., & Meyer, M.
Affiliations
  • University of Southern Denmark (DK)
  • University of Copenhagen (DK)
Corresponding author
Meyer, Morten
Venue
Cells
Publisher
MDPI AG
Published
2022-05-01
Volume
11
Issue
9
Pages
1520
ISSN
2073-4409 (ISSN-L)
OpenAlex
W4225258118
Cited by
110
References
233
Citations by year
2026: 20 · 2025: 24 · 2024: 32 · 2023: 28 · 2022: 6
FWCI
2.34
Type
journal article
Language
en
Open access
Locations
  • Cells (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • Research at the University of Copenhagen (University of Copenhagen) (publishedVersion, cc-by)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
  • University of Southern Denmark Research Portal (University of Southern Denmark) (submittedVersion, cc-by)
Funders
  • Innovation Fund Denmark (4108-00008A)
  • the Danish Parkinson Foundation, the Jascha Foundation (4108-00008A)
  • the Faculty of Health Sciences at the University of Southern Denmark (4108-00008A)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Protein Kinase Regulation and GTPase Signaling
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
Keywords
RhoA, Rho GTPases, neurodegenerative diseases, Parkinson's disease, Alzheimer's disease, Huntington's disease, amyotrophic lateral sclerosis, RhoA signaling, RhoA inhibition, therapeutic target, neurons, glial cells, signal transduction
MeSH terms
Humans, Huntington Disease, Neurons, Parkinson Disease, Signal Transduction, Neurodegenerative Diseases, rhoA GTP-Binding Protein

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