Table 2.

STING signaling in neurological disease

Neurological diseaseType of evidence for STING involvementMode of STING activationRole of STING signaling
ALS and FTD Genetic, pharmacological inhibitor Ligand (mtDNA, e.g., TDP-43), trafficking (e.g., C9ORF72) Genetic deficiency or inhibition of STING signaling in a mouse model protects against disease progression; STING inhibitor reduces IFN expression in patient cells (Yu et al., 2020; McCauley et al., 2020) 
PD Genetic, expression Ligand (mtDNA) Protection from development of motor deficits in a STING-deficient mouse model; STING protein expression in substantia nigra correlates with α-synuclein deposition in patients (Hinkle et al., 2022; Sliter et al., 2018) 
NPC Genetic Trafficking STING-deficient mouse model displays improved motor function (Chu et al., 2021) 
Huntington’s disease Expression Unknown Elevated p-STING in a mouse model (Sharma et al., 2020) 
Ataxia telangiectasia Pharmacological inhibitor Ligand (likely gDNA from DNA damage) Pharmacological inhibition of STING reduces senescence phenotypes and inflammation in brain organoids model (Aguado et al., 2021) 
Traumatic brain injury Genetic, expression Unknown Decreased levels of pro-inflammatory cytokines in a STING-deficient mouse model; STING expression and activity upregulated in late trauma human brain samples and a mouse model (Abdullah et al., 2018; Barrett et al., 2020; Sen et al., 2020) 
Ischemic brain injury and stroke Pharmacological inhibitor Unknown Pharmacological inhibition of STING reduces brain infarction, neuronal injury, and cognitive deficits in a mouse model (Kong et al., 2022) 
Subarachnoid hemorrhage Pharmacological inhibitor Unknown STING inhibition improves edema, neuronal injury, inflammation, and behavioral outcome in a mouse model (Peng et al., 2020) 
Neurological diseaseType of evidence for STING involvementMode of STING activationRole of STING signaling
ALS and FTD Genetic, pharmacological inhibitor Ligand (mtDNA, e.g., TDP-43), trafficking (e.g., C9ORF72) Genetic deficiency or inhibition of STING signaling in a mouse model protects against disease progression; STING inhibitor reduces IFN expression in patient cells (Yu et al., 2020; McCauley et al., 2020) 
PD Genetic, expression Ligand (mtDNA) Protection from development of motor deficits in a STING-deficient mouse model; STING protein expression in substantia nigra correlates with α-synuclein deposition in patients (Hinkle et al., 2022; Sliter et al., 2018) 
NPC Genetic Trafficking STING-deficient mouse model displays improved motor function (Chu et al., 2021) 
Huntington’s disease Expression Unknown Elevated p-STING in a mouse model (Sharma et al., 2020) 
Ataxia telangiectasia Pharmacological inhibitor Ligand (likely gDNA from DNA damage) Pharmacological inhibition of STING reduces senescence phenotypes and inflammation in brain organoids model (Aguado et al., 2021) 
Traumatic brain injury Genetic, expression Unknown Decreased levels of pro-inflammatory cytokines in a STING-deficient mouse model; STING expression and activity upregulated in late trauma human brain samples and a mouse model (Abdullah et al., 2018; Barrett et al., 2020; Sen et al., 2020) 
Ischemic brain injury and stroke Pharmacological inhibitor Unknown Pharmacological inhibition of STING reduces brain infarction, neuronal injury, and cognitive deficits in a mouse model (Kong et al., 2022) 
Subarachnoid hemorrhage Pharmacological inhibitor Unknown STING inhibition improves edema, neuronal injury, inflammation, and behavioral outcome in a mouse model (Peng et al., 2020) 

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