Physiological and pathological functions of neuroserpin: Regulation of cellular responses through multiple mechanisms

Lee, Tet Woo; Tsang, Vicky W. K.; Loef, Evert Jan; Birch, Nigel P.

doi:10.1016/j.semcdb.2016.09.007

Cited by 31 publications

(25 citation statements)

References 82 publications

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“…Because of the short half‐life of this molecule (~ 10 min, Lee et al. ) its constitutive expression may be vital in blocking the activation of the proteolytic cascade in the CNS.…”

Section: Discussionmentioning

confidence: 99%

“…Neuroserpin binds tissue plasminogen activator, blocking the conversion of plasminogen to plasmin and thus inhibiting the proteolytic cascade (Vivien & Buisson, ; Lee et al. ). This function makes neuroserpin a promising therapeutic target in conditions where the proteolytic homeostasis of the CNS is impaired, such as hypoxia‐ischaemia (Millar et al.…”

Section: Introductionmentioning

confidence: 99%

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Neuroserpin expression during human brain development and in adult brain revealed by immunohistochemistry and single cell RNA sequencing

et al. 2019

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Neuroserpin is a serine‐protease inhibitor mainly expressed in the CNS and involved in the inhibition of the proteolytic cascade. Animal models confirmed its neuroprotective role in perinatal hypoxia‐ischaemia and adult stroke. Although neuroserpin may be a potential therapeutic target in the treatment of the aforementioned conditions, there is still no information in the literature on its distribution during human brain development. The present study provides a detailed description of the changing spatiotemporal patterns of neuroserpin focusing on physiological human brain development. Five stages were distinguished within our examined age range which spanned from the 7th gestational week until adulthood. In particular, subplate and deep cortical plate neurons were identified as the main sources of neuroserpin production between the 25th gestational week and the first postnatal month. Our immunohistochemical findings were substantiated by single cell RNA sequencing data showing specific neuronal and glial cell types expressing neuroserpin. The characterization of neuroserpin expression during physiological human brain development is essential for forthcoming studies which will explore its involvement in pathological conditions, such as perinatal hypoxia‐ischaemia and adult stroke in human.

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“…Because of the short half‐life of this molecule (~ 10 min, Lee et al. ) its constitutive expression may be vital in blocking the activation of the proteolytic cascade in the CNS.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Neuroserpin expression during human brain development and in adult brain revealed by immunohistochemistry and single cell RNA sequencing

et al. 2019

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“…The clinical features of this neuroserpinopathy could be related either to a toxic effect of the polymers that accumulate within the endoplasmic reticulum of neurons, to the lack of intact extracellular neuroserpin or a combination of both (Miranda and Lomas, ). Loss of extracellular neuroserpin may account for seizure progression and neuron degeneration via a non‐inhibited tPA activity pathway (Lee, Tsang, Loef, & Birch, ). G392E and G392R variants are associated with the more severe phenotypes described so far (Davis et al, ).…”

Section: Discussionmentioning

confidence: 99%

SERPINI1 pathogenic variants: An emerging cause of childhood‐onset progressive myoclonic epilepsy

Ranza

Garcia-Tarodo

Varvagiannis

et al. 2017

American J of Med Genetics Pt A

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Progressive myoclonic epilepsies are rare neurodegenerative diseases with a wide spectrum of clinical presentations and genetic heterogeneity that render their diagnosis perplexing. Discovering new imputable genes has been an ongoing process in recent years. We present two pediatric cases of progressive myoclonic epilepsy with SERPINI1 pathogenic variants that lead to a severe presentation; we highlight the importance of including this gene, previously known as causing an adult-onset dementia-epilepsy syndrome, in the genetic work-up of childhood-onset progressive myoclonic epilepsies.

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“…Neuroserpin also belongs to the serpin family of serine protease inhibitors, and is expressed mainly in the central nervous system, where it inhibits tPA, thus protecting neurons from the cytotoxic effect of plasmin [ 15 , 16 ]. Apart from the study by Valiente et al, expression levels of Neuroserpin has not been studied in human cancer [ 17 ], and the exact role of Neuroserpin in cancer and metastasis remains to be elucidated.…”

Section: Introductionmentioning

confidence: 99%

Low expression of SerpinB2 is associated with reduced survival in lung adenocarcinomas

et al. 2017

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Physiological and pathological functions of neuroserpin: Regulation of cellular responses through multiple mechanisms

Cited by 31 publications

References 82 publications

Neuroserpin expression during human brain development and in adult brain revealed by immunohistochemistry and single cell RNA sequencing

Neuroserpin expression during human brain development and in adult brain revealed by immunohistochemistry and single cell RNA sequencing

SERPINI1 pathogenic variants: An emerging cause of childhood‐onset progressive myoclonic epilepsy

Low expression of SerpinB2 is associated with reduced survival in lung adenocarcinomas

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