Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum

The neuronal ceroid lipofuscinoses (NCLs) are a group of devastating neurological disorders that have a global distribution and affect people of all ages. Commonly known as Batten disease, this form of neurodegeneration is linked to mutations in 13 genetically distinct genes. The precise mechanisms...

Full description

Saved in:
Bibliographic Details
Published in:Cells (Basel, Switzerland) Switzerland), 2019-02, Vol.8 (2), p.115
Main Authors: McLaren, Meagan D, Mathavarajah, Sabateeshan, Huber, Robert J
Format: Article
Language:English
Subjects:
Alzheimer's disease
Autophagy
Batten disease
Cell cycle
Cell growth
CLN3
CLN5
development
Dictyostelium
Dictyostelium - metabolism
Dictyostelium discoideum
Enzymes
Fibroblasts
Genes
Genomes
Homeostasis
Humans
Life cycles
Lipids
Localization
Models, Biological
Morphology
Mutation
Neurodegeneration
Neurological diseases
Neuronal ceroid lipofuscinosis
Neuronal Ceroid-Lipofuscinoses - metabolism
Nutrients
Osmoregulation
Phenotype
Proteins
Protozoan Proteins - metabolism
Review
Signal transduction
Stem cells
TPP1/CLN2
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:The neuronal ceroid lipofuscinoses (NCLs) are a group of devastating neurological disorders that have a global distribution and affect people of all ages. Commonly known as Batten disease, this form of neurodegeneration is linked to mutations in 13 genetically distinct genes. The precise mechanisms underlying the disease are unknown, in large part due to our poor understanding of the functions of NCL proteins. The social amoeba has proven to be an exceptional model organism for studying a wide range of neurological disorders, including the NCLs. The genome contains homologs of 11 of the 13 NCL genes. Its life cycle, comprised of both single-cell and multicellular phases, provides an excellent system for studying the effects of NCL gene deficiency on conserved cellular and developmental processes. In this review, we highlight recent advances in NCL research using as a biomedical model.
ISSN:2073-4409
2073-4409
DOI:10.3390/cells8020115