Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.

Neurobiology of disease - 1 Jan 2007

Kielar Catherine, Maddox Lucy, Bible Ellen, Pontikis Charlie C, Macauley Shannon L, Griffey Megan A, Wong Michael, Sands Mark S, Cooper Jonathan D

Abstract excerpt

Infantile neuronal ceroid lipofuscinosis (INCL) is caused by deficiency of the lysosomal enzyme, palmitoyl protein thioesterase 1 (PPT1). We have investigated the onset and progression of pathological changes in Ppt1 deficient mice (Ppt1-/-) and the development of their seizure phenotype. Surprisingly, cortical atrophy and neuron loss occurred only late in disease progression but were preceded by localized...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Astrocytes
Share to Atrophy

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Gliosis

Share to Interneurons

Share to Neuroglia

Share to Neuronal Ceroid-Lipofuscinoses

Share to Thalamus

Share to Thiolester Hydrolases

Source

Open in PubMed Open full text

Identifiers

PMID: 17046272
DOI: 10.1016/j.nbd.2006.09.001
PMCID: PMC1866219

Genes

PPT1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.PMID 17046272Read ↗

The Role of Attenuated Astrocyte Activation in Infantile Neuronal Ceroid LipofuscinosisSemantic proximity Read ↗

Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons.Semantic proximity Read ↗

Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.Semantic proximity Read ↗

Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe?Semantic proximity Read ↗

Decreased sensitivity of palmitoyl protein thioesterase 1-deficient neurons to chemical anoxia.Semantic proximity Read ↗

NMDA Receptor Dysregulation by Defective Depalmitoylation in the Infantile Neuronal Ceroid Lipofuscinosis Mouse ModelSemantic proximity Read ↗

The Batten disease Palmitoyl Protein Thioesterase 1 gene regulates neural specification and axon connectivity during Drosophila embryonic development.Semantic proximity Read ↗

Molecular correlates of axonal and synaptic pathology in mouse models of Batten diseaseSemantic proximity Read ↗

Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice.Semantic proximity Read ↗

Ocular phenotype in a mouse gene knockout model for infantile neuronal ceroid lipofuscinosis.Semantic proximity Read ↗

Palmitoyl-protein thioesterase 1 deficiency in Drosophila melanogaster causes accumulation of abnormal storage material and reduced life span.Semantic proximity Read ↗

The novel Cln1R151X mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapySemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.