Bilirubin encephalopathy

S Qian, P Kumar, FD Testai - Current Neurology and Neuroscience …, 2022 - Springer
Abstract Purpose of Review Hyperbilirubinemia is commonly seen in neonates. Though
hyperbilirubinemia is typically asymptomatic, severe elevation of bilirubin levels can lead to …

Review of bilirubin neurotoxicity II: preventing and treating acute bilirubin encephalopathy and kernicterus spectrum disorders

SM Shapiro, SM Riordan - Pediatric research, 2020 - nature.com
Previously in Part I of this two-part review, we discussed the current and recent advances in
the understanding of the molecular biology and neuropathology of bilirubin neurotoxicity …

Promoterless gene targeting without nucleases rescues lethality of a Crigler‐Najjar syndrome mouse model

F Porro, G Bortolussi, A Barzel… - EMBO molecular …, 2017 - embopress.org
Crigler‐Najjar syndrome type I (CNSI) is a rare monogenic disease characterized by severe
neonatal unconjugated hyperbilirubinemia with a lifelong risk of neurological damage and …

Bilirubin‐induced oxidative stress leads to DNA damage in the cerebellum of hyperbilirubinemic neonatal mice and activates DNA double‐strand break repair …

V Rawat, G Bortolussi, S Gazzin… - … medicine and cellular …, 2018 - Wiley Online Library
Unconjugated bilirubin is considered a potent antioxidant when present at moderate levels.
However, at high concentrations, it produces severe neurological damage and death …

[HTML][HTML] Coupling AAV-mediated promoterless gene targeting to SaCas9 nuclease to efficiently correct liver metabolic diseases

A De Caneva, F Porro, G Bortolussi, R Sola, M Lisjak… - JCI insight, 2019 - ncbi.nlm.nih.gov
Nonintegrative AAV-mediated gene therapy in the liver is effective in adult patients but faces
limitations in pediatric settings because of episomal DNA loss during hepatocyte …

Neuroinflammation and ER-stress are key mechanisms of acute bilirubin toxicity and hearing loss in a mouse model

E Schiavon, JL Smalley, S Newton, NH Greig… - PLoS …, 2018 - journals.plos.org
Hyperbilirubinemia (jaundice) is caused by raised levels of unconjugated bilirubin in the
blood. When severe, susceptible brain regions including the cerebellum and auditory …

Maternal linoleic acid-rich diet ameliorates bilirubin neurotoxicity in offspring mice

D Yan, XT Wu, X Chen, J Wang, F Ge, M Wu, J Wu… - Cell Death …, 2024 - nature.com
Hyperbilirubinaemia is a prevalent condition during the neonatal period, and if not promptly
and effectively managed, it can lead to severe bilirubin-induced neurotoxicity. Sunflower …

A translationally optimized AAV-UGT1A1 vector drives safe and long-lasting correction of Crigler-Najjar syndrome

G Ronzitti, G Bortolussi, R Van Dijk, F Collaud… - … therapy Methods & …, 2016 - cell.com
Crigler-Najjar syndrome is a severe metabolic disease of the liver due to a reduced activity
of the UDP Glucuronosyltransferase 1A1 (UGT1A1) enzyme. In an effort to translate to the …

Attenuation of neuro-inflammation improves survival and neurodegeneration in a mouse model of severe neonatal hyperbilirubinemia

S Vodret, G Bortolussi, A Iaconcig, E Martinelli… - Brain, behavior, and …, 2018 - Elsevier
All pre-term newborns and a high proportion of term newborns develop neonatal jaundice.
Neonatal jaundice is usually a benign condition and self-resolves within few days after birth …

Models of bilirubin neurological damage: lessons learned and new challenges

S Gazzin, S Jayanti, C Tiribelli - Pediatric Research, 2023 - nature.com
Objective Jaundice (icterus) is the visible manifestation of the accumulation of bilirubin in the
tissue and is indicative of potential toxicity to the brain. Since its very first description more …