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

Thymic involution and corticosterone level in Sandhoff disease model mice: new aspects the pathogenesis of GM2 gangliosidosis.

Journal of inherited metabolic disease - 1 Oct 2011

Matsuoka Kazuhiko, Tsuji Daisuke, Taki Takao, Itoh Kohji

Abstract excerpt

Sandhoff disease (SD) is a lysosomal disease caused by a mutation of the HEXB gene associated with excessive accumulation of GM2 ganglioside (GM2) in lysosomes and neurological manifestations. Production of autoantibodies against the accumulated gangliosides has been reported to be involved in the progressive pathogenesis of GM2 gangliosidosis, although the underlying mechanism has not been fully elucidated. The...

Read the complete abstract on PubMed

Topics

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

Share to Age Factors
Share to Atrophy
Share to Caspases

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Corticosterone

Share to Disease Progression

Share to G(M2) Ganglioside

Share to Sandhoff Disease

Share to Thymus Gland

Source

Identifiers

PMID: 21598013
DOI: 10.1007/s10545-011-9316-6

Genes

HEXB

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. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Thymic involution and corticosterone level in Sandhoff disease model mice: new aspects the pathogenesis of GM2 gangliosidosis.PMID 21598013Read ↗

Apoptotic Cell Death in Mouse Models of GM2 Gangliosidosis and Observations on Human Tay-Sachs and Sandhoff DiseasesSemantic proximity Read ↗

GM2 ganglioside accumulation causes neuroinflammation and behavioral alterations in a mouse model of early-onset Tay-Sachs disease.Semantic proximity Read ↗

FcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model mice.Semantic proximity Read ↗

Possible role of autoantibodies in the pathophysiology of GM2 gangliosidosesSemantic proximity Read ↗

Possible role of autoantibodies in the pathophysiology of GM2 gangliosidosesSemantic proximity Read ↗

[Molecular pathogenesis and therapeutic approach of GM2 gangliosidosis].Semantic proximity Read ↗

Time course of cytokine upregulation in the lacrimal gland and presence of autoantibodies in a predisposed mouse model of Sjögren's Syndrome: the influence of sex hormones and genetic background.Semantic proximity Read ↗

Cell Loss and Autophagy in the Extra‐Adrenal Chromaffin Organ of Zuckerkandl are Regulated by Glucocorticoid SignallingSemantic proximity Read ↗

Mast cell hyperplasia in the skin of Dsg4-deficient hypotrichosis mice, which are long-living mutants of lupus-prone mice.Semantic proximity Read ↗

Thymic alterations in feline GM1 gangliosidosis.Semantic proximity Read ↗

Fragmentation of Golgi complex and Golgi autoantigens during apoptosis and necrosisSemantic proximity Read ↗

Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.Semantic proximity Read ↗

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