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Updated: Jun 13, 2026

A Neonatal BALB/c Mouse Model of Necrotizing Enterocolitis
05:39

A Neonatal BALB/c Mouse Model of Necrotizing Enterocolitis

Published on: November 30, 2021

[NCL in animal models].

K Rüther1

  • 1Arbeitsbereich Strabologie/Neuroophthalmologie, Augenklinik, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany. Klaus.ruether@charite.de

Der Ophthalmologe : Zeitschrift Der Deutschen Ophthalmologischen Gesellschaft
|May 11, 2010
PubMed
Summary
This summary is machine-generated.

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Neuronal ceroid lipofuscinoses (NCL) are fatal neurodegenerative lysosomal storage diseases. Mouse models are crucial for studying NCL, but their retinal phenotypes can differ from human patients.

Area of Science:

  • Neurology
  • Genetics
  • Cell Biology

Context:

  • Neuronal ceroid lipofuscinoses (NCL) represent a group of severe, fatal neurodegenerative lysosomal storage diseases.
  • Characterized by progressive neurological decline, including seizures, dementia, and motor deficits, NCL leads to premature death.
  • Currently, 10 subtypes are recognized, with genetic defects identified in eight, involving various soluble and membrane proteins of largely unknown function.

Purpose:

  • To highlight the critical role of animal models, particularly mouse models, in investigating the pathology and pathophysiology of NCL.
  • To underscore the challenges posed by discrepancies in retinal phenotypes between mouse models and human patients.
  • To emphasize the rapid translation of therapeutic strategies from mouse models to human clinical trials due to the severity of NCL.

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Use of Animal Model of Sepsis to Evaluate Novel Herbal Therapies
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Published on: April 11, 2012

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Last Updated: Jun 13, 2026

A Neonatal BALB/c Mouse Model of Necrotizing Enterocolitis
05:39

A Neonatal BALB/c Mouse Model of Necrotizing Enterocolitis

Published on: November 30, 2021

Use of Animal Model of Sepsis to Evaluate Novel Herbal Therapies
07:34

Use of Animal Model of Sepsis to Evaluate Novel Herbal Therapies

Published on: April 11, 2012

Summary:

  • NCL are devastating neurodegenerative lysosomal storage diseases with significant clinical manifestations.
  • Research heavily relies on mouse models for understanding NCL, yet phenotypic variations, especially in the retina, can complicate therapeutic assessments.
  • The urgent need for treatments drives swift clinical application of findings from preclinical models.

Impact:

  • Facilitates a deeper understanding of NCL pathogenesis and progression.
  • Informs the development and refinement of preclinical models for NCL research.
  • Accelerates the translation of potential therapies for NCL patients, offering hope for improved outcomes.