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

A molecular investigation of true dominance in Huntington's disease.

Journal of medical genetics - 1 Oct 1999

Narain Y, Wyttenbach A, Rankin J, Furlong R A, Rubinsztein D C

Abstract excerpt

Huntington's disease (HD) is thought to show true dominance, since subjects with two mutant alleles have been reported to have similar ages at onset of disease compared to heterozygous sibs. We have investigated this phenomenon using a cell culture model. Protein aggregate formation was used as an indicator for pathology, as intraneuronal huntingtin inclusions are associated with pathology in vitro and in vivo....

Read the complete abstract on PubMed

Topics

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

Share to COS Cells
Share to Dose-Response Relationship, Drug
Share to Genes, Dominant

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

Share to Huntingtin Protein

Share to Huntington Disease

Share to Mutagenesis

Share to Nerve Tissue Proteins

Share to Trinucleotide Repeats

Source

Open in PubMed Open full text

Identifiers

PMID: 10528852
DOI: 10.1136/jmg.36.10.739
PMCID: PMC1734229

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

A molecular investigation of true dominance in Huntington's disease.PMID 10528852Read ↗

Huntington's disease: from pathology and genetics to potential therapies.Semantic proximity Read ↗

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.Semantic proximity Read ↗

An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity.Semantic proximity Read ↗

Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington’S Disease ModelSemantic proximity Read ↗

A cellular model that recapitulates major pathogenic steps of Huntington's disease.Semantic proximity Read ↗

Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease.Semantic proximity Read ↗

Huntingtin aggregation and toxicity in Huntington's diseaseSemantic proximity Read ↗

Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course.Semantic proximity Read ↗

HD mutation results in a dominant negative effect on HTT functionSemantic proximity Read ↗

Formation of Polyglutamine Inclusions in Non-CNS TissueSemantic proximity Read ↗

Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein.Semantic proximity Read ↗

In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.Semantic proximity Read ↗

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