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

Altered tryptophan metabolism in the brain of cystatin B-deficient mice: a model system for progressive myoclonus epilepsy.

Epilepsia - 1 Oct 2006

Vaarmann Annika, Kaasik Allen, Zharkovsky Alexander

Abstract excerpt

PURPOSE: Progressive myoclonus epilepsy of the Unverricht-Lundborg type (EPM1) is a rare neurologic disorder, associated with mutations in the Cystatin B (Cstb) gene. Mice lacking Cstb, a cysteine protease inhibitor of the cathepsine family of proteases, provide a mammalian model for EPM1 by displaying similarly progressive ataxia, myoclonic seizures, and neurodegeneration. However, the linkage of Cstb deficit on...

Read the complete abstract on PubMed

Topics

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

Share to Cerebellum
Share to Cerebral Cortex
Share to Cystatins

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Hydroxyindoleacetic Acid

Share to Kynurenine

Share to Serotonin

Share to Synaptic Transmission

Share to Tryptophan

Source

Identifiers

PMID: 17054687
DOI: 10.1111/j.1528-1167.2006.00638.x

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

Altered tryptophan metabolism in the brain of cystatin B-deficient mice: a model system for progressive myoclonus epilepsy.PMID 17054687Read ↗

A pathogenetic hypothesis of Unverricht-Lundborg disease onset and progression.Semantic proximity Read ↗

Quantitative Changes in the Mitochondrial Proteome of Cerebellar Synaptosomes From Preclinical Cystatin B-Deficient MiceSemantic proximity Read ↗

Neuropathological changes in a mouse model of progressive myoclonus epilepsy: cystatin B deficiency and Unverricht-Lundborg disease.Semantic proximity Read ↗

Progressive ataxia, myoclonic epilepsy and cerebellar apoptosis in cystatin B-deficient mice.Semantic proximity Read ↗

Cystatin B: mutation detection, alternative splicing and expression in progressive myclonus epilepsy of Unverricht-Lundborg type (EPM1) patients.Semantic proximity Read ↗

Cystatin B-deficient mice have increased expression of apoptosis and glial activation genesSemantic proximity Read ↗

The epilepsy, the protease inhibitor and the dodecamer: progressive myoclonus epilepsy, cystatin b and a 12-mer repeat expansion.Semantic proximity Read ↗

Seizures, ataxia, and neuronal loss in cystatin B heterozygous mice.Semantic proximity Read ↗

Unverricht-Lundborg disease with cystatin B gene abnormalities.Semantic proximity Read ↗

The ACMSD gene, involved in tryptophan metabolism, is mutated in a family with cortical myoclonus, epilepsy, and parkinsonism.Semantic proximity Read ↗

Mutations in the Gene Encoding Cystatin B in Progressive Myoclonus Epilepsy (EPM1)Semantic proximity Read ↗

Behavioral Balance in Tryptophan Turmoil: Regional Metabolic Rewiring in Kynurenine Aminotransferase II Knockout MiceSemantic proximity Read ↗

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