Infantile neuronal ceroid lipofuscinosis (INCL) is one of a group of fatal hereditary lysosomal storage disorders. Palmitoyl protein thioesterase 1 null mutant mice (PPT1-/-) now exist that accurately recapitulate many important disease features. The severely affected PPT1-/- mouse CNS exhibited reduced volume of both cortical and subcortical regions, but with sparing of the cerebellum. Pronounced differences existed in the extent of cortical thinning between different regions, due to lamina-specific effects upon neuronal survival. A dramatic reduction in cortical and hippocampal interneuron number was also evident, with different extents of specific interneuron loss depending upon the region and phenotypic marker. These neuronal changes were accompanied by widespread astrocytosis and localized microglial activation in restricted cortical and subcortical regions. This characterization of PPT1-/- mice not only provides defined pathological landmarks for understanding disease pathogenesis, but also provides an invaluable resource for subsequently judging the efficacy of therapeutic strategies.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.nbd.2004.02.010 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Protein palmitoylation in hepatic diseases: Functional insights and therapeutic strategies.
J Adv Res
December 2024
The First Affiliated Hospital of Xinxiang Medical University, Xinxiang, Henan, PR China; Institute of Psychiatry and Neuroscience of Xinxiang Medical University, Xinxiang, Henan, PR China; Laboratory of Genetic Regulators in the Immune System, School of Medical Technology, Xinxiang Medical University, Xinxiang, Henan, PR China. Electronic address:
Background: Liver pathologies represent a spectrum of conditions ranging from fatty liver to the aggressive hepatocellular carcinoma (HCC), as well as parasitic infections, which collectively pose substantial global health challenges. S-palmitoylation (commonly referred to as palmitoylation), a post-translational modification (PTM) characterized by the covalent linkage of a 16-carbon palmitic acid (PA) chain to specific cysteine residues on target proteins, plays a pivotal role in diverse cellular functions and is intimately associated with the liver's physiological and pathological states.
Aim Of Review: This study aims to elucidate how protein palmitoylation affects liver disease pathophysiology and evaluates its potential as a target for diagnostic and therapeutic interventions.
The blue-light receptor CRY1 serves as a switch to balance photosynthesis and plant defense.
Cell Host Microbe
December 2024
CAS Center for Excellence in Molecular Plant Sciences (CEMPS), Institute of Plant Physiology and Ecology (SIPPE), Chinese Academy of Sciences, Shanghai 200031, People's Republic of China; College of Life Sciences and Oceanography, Shenzhen University, Shenzhen 518060, People's Republic of China. Electronic address:
Plant stomata open in response to blue light, allowing gas exchange and water transpiration. However, open stomata are potential entry points for pathogens. Whether plants can sense pathogens and mount defense responses upon stomatal opening and how blue-light cues are integrated to balance growth-defense trade-offs are poorly characterized.
View Article and Find Full Text PDFUnderstanding Post-Translational Modifications in Porcine Reproductive and Respiratory Syndrome Virus Infection.
Vet Sci
December 2024
Pingliang Vocational and Technical College, Pingliang 744000, China.
Porcine reproductive and respiratory syndrome virus (PRRSV) is a highly contagious virus affecting pigs with significant impacts to the swine industry worldwide. This review provides a comprehensive understanding of post-translational modifications (PTMs) associated with PRRSV infection. We discuss the various types of PTMs, including phosphorylation, ubiquitination, SUMoylation, acetylation, glycosylation, palmitoylation, and lactylation, that occur during PRRSV infection.
View Article and Find Full Text PDFRecapitulating the potential contribution of protein S-palmitoylation in cancer.
Cancer Metastasis Rev
December 2024
Department of Biosciences and Biomedical Engineering, Indian Institute of Technology Indore, Khandwa Road, 453552, Simrol, Madhya Pradesh, India.
Protein S-palmitoylation is a reversible form of protein lipidation in which the formation of a thioester bond occurs between a cysteine (Cys) residue of a protein and a 16-carbon fatty acid chain. This modification is catalyzed by a family of palmitoyl acyl transferases, the DHHC enzymes, so called because of their Asp-His-His-Cys (DHHC) catalytic motif. Deregulation of DHHC enzymes has been linked to various diseases, including cancer and infections.
View Article and Find Full Text PDFMitochondrial fatty acid oxidase CPT1A ameliorates postoperative cognitive dysfunction by regulating astrocyte ferroptosis.
Brain Res
December 2024
Department of Urology Surgery, People's Hospital of Shenzhen, Shenzhen City, Guangdong Province, China.
Article Synopsis
- Postoperative cognitive dysfunction (POCD) is a complication related to surgery involving cognitive decline, potentially influenced by neuroinflammation, ferroptosis, and mitochondrial fatty acid metabolism, particularly via the protein Carnitine palmitoyl transferase 1a (CPT1A).
- The study used SVG P12 astrocytes to explore how CPT1A affects mitochondrial function, inflammation, and neuron damage by overexpressing or knocking down CPT1A and assessing the resulting changes in cellular conditions.
- Results showed that higher levels of CPT1A improved mitochondrial function and reduced oxidative stress and inflammation in astrocytes, which benefitted neuron health, while these effects depended on functional mitochondrial respiration, as demonstrated when treatments disrupted this process.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!