Neuroserpin, a crucial regulator for axogenesis, synaptic modelling and cell–cell interactions in the pathophysiology of neurological disease

A Godinez, R Rajput, N Chitranshi, V Gupta… - Cellular and Molecular …, 2022 - Springer
Neuroserpin is an axonally secreted serpin that is involved in regulating plasminogen and
its enzyme activators, such as tissue plasminogen activator (tPA). The protein has been …

Disruption of the ubiquitin-proteasome system and elevated endoplasmic reticulum stress in epilepsy

S Poliquin, JQ Kang - Biomedicines, 2022 - mdpi.com
The epilepsies are a broad group of conditions characterized by repeated seizures, and
together are one of the most common neurological disorders. Additionally, epilepsy is …

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

TW Lee, VWK Tsang, EJ Loef, NP Birch - Seminars in cell & developmental …, 2017 - Elsevier
It is 27 years since neuroserpin was first discovered in the nervous system and identified as
a member of the serpin superfamily. Since that time potential roles for this serine protease …

Icariin induces synoviolin expression through NFE2L1 to protect neurons from ER stress-induced apoptosis

F Li, B Gao, H Dong, J Shi, D Fang - PloS one, 2015 - journals.plos.org
By suppressing neuronal apoptosis, Icariin is a potential therapeutic drug for neuronal
degenerative diseases. The molecular mechanisms of Icariin anti-apoptotic functions are still …

Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB

NA Guadagno, C Moriconi, V Licursi, E D'Acunto… - Neurobiology of …, 2017 - Elsevier
The serpinopathies are human pathologies caused by mutations that promote
polymerisation and intracellular deposition of proteins of the serpin superfamily, leading to a …

Polymerogenic neuroserpin causes mitochondrial alterations and activates NFκB but not the UPR in a neuronal model of neurodegeneration FENIB

E D'Acunto, L Gianfrancesco, I Serangeli… - Cellular and Molecular …, 2022 - Springer
The neurodegenerative condition FENIB (familiar encephalopathy with neuroserpin
inclusion bodies) is caused by heterozygous expression of polymerogenic mutant …

[HTML][HTML] The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin

JA Dickens, A Ordóñez, JE Chambers… - The FASEB …, 2016 - ncbi.nlm.nih.gov
Abstract α 1-Antitrypsin is a serine protease inhibitor produced in the liver that is responsible
for the regulation of pulmonary inflammation. The commonest pathogenic gene mutation …

New concepts in alpha-1 antitrypsin deficiency disease mechanisms

SJ Marciniak, A Ordóñez, JA Dickens… - Annals of the …, 2016 - atsjournals.org
Alpha-1 antitrypsin deficiency is predominantly caused by point mutations that alter the
protein's folding. These mutations fall into two broad categories: those that destabilize the …

UBE2J1 is identified as a novel plasma cell-related gene involved in the prognosis of high-grade serous ovarian cancer

Y Tian, R Dong, Y Guan, Y Wang, W Zhao… - Journal of Translational …, 2025 - Springer
Background Immune cells within tumor tissues play important roles in remodeling the tumor
microenvironment, thus affecting tumor progression and the therapeutic response. The …

Interactions between N‐linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum

C Moriconi, A Ordonez, G Lupo, B Gooptu… - The FEBS …, 2015 - Wiley Online Library
The neuronal serpin neuroserpin undergoes polymerisation as a consequence of point
mutations that alter its conformational stability, leading to a neurodegenerative dementia …