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

Lysosomal storage results in impaired survival but normal neurite outgrowth in dorsal root ganglion neurones from a mouse model of Sandhoff disease.

Neuropathology and applied neurobiology - 1 Feb 2002

Sango K, Yamanaka S, Ajiki K, Tokashiki A, Watabe K

Abstract excerpt

Sandhoff disease is a heritable lysosomal storage disease resulting from impaired degradation of GM2 ganglioside and related substrates. A mouse model of Sandhoff disease created by gene targeting displays progressive neurological manifestations, similar to patients with the disease. In the present in vivo and in vitro studies, we examined morphological and functional abnormalities of dorsal root ganglion (DRG)...

Read the complete abstract on PubMed

Topics

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

Share to G(M2) Ganglioside
Share to Ganglia, Spinal

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to In Vitro Techniques

Share to Inclusion Bodies

Share to Lysosomes

Share to Microscopy, Electron

Share to Neurites

Share to Sandhoff Disease

Source

Identifiers

PMID: 11849560
DOI: 10.1046/j.1365-2990.2002.00366.x

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

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

Lysosomal storage results in impaired survival but normal neurite outgrowth in dorsal root ganglion neurones from a mouse model of Sandhoff disease.PMID 11849560Read ↗

Murine models of acute neuronopathic Gaucher diseaseSemantic proximity Read ↗

Adeno-associated virus-mediated expression of β-hexosaminidase prevents neuronal loss in the Sandhoff mouse brainSemantic proximity Read ↗

Murine Models of Lysosomal Storage Diseases Exhibit Differences in Brain Protein Aggregation and NeuroinflammationSemantic proximity Read ↗

Establishment of immortalized Schwann cells from Sandhoff mice and corrective effect of recombinant human beta-hexosaminidase A on the accumulated GM2 ganglioside.Semantic proximity Read ↗

Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease.Semantic proximity Read ↗

Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with <i>N</i> -butyldeoxynojirimycinSemantic proximity Read ↗

Loss of TMEM106B and PGRN leads to severe lysosomal abnormalities and neurodegeneration in miceSemantic proximity Read ↗

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism.Semantic proximity Read ↗

Ultrastructural analysis of neuronal and non-neuronal lysosomal storage in mucolipidosis type II knock-in mice.Semantic proximity Read ↗

LAMP-2 deficiency leads to hippocampal dysfunction but normal clearance of neuronal substrates of chaperone-mediated autophagy in a mouse model for Danon diseaseSemantic proximity Read ↗

Viable Neuronopathic Gaucher Disease Model in Medaka (Oryzias latipes) Displays Axonal Accumulation of Alpha-SynucleinSemantic proximity Read ↗

Altered expression and distribution of cathepsins in neuronopathic forms of Gaucher disease and in other sphingolipidosesSemantic proximity Read ↗

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