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Article

Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models.

Journal of neuropathology and experimental neurology - 1 Apr 2007

Schilling Gabriele, Klevytska Alexandra, Tebbenkamp Andrew T N, Juenemann Katrin, Cooper Jillian, Gonzales Victoria, Slunt Hilda, Poirer Michelle, Ross Christopher A, Borchelt David R

Abstract excerpt

Huntington disease (HD) is caused by the expansion of a glutamine (Q) repeat near the N terminus of huntingtin (htt), resulting in altered conformation of the mutant protein to produce, most prominently in brain neurons, nuclear and cytoplasmic inclusion pathology. The inclusions and associated diffuse accumulation of mutant htt in nuclei are composed of N-terminal fragments of mutant protein. Here, we used a...

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PMID: 17413322
DOI: 10.1097/nen.0b013e318040b2c8

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

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