Huntington's disease (HD) is a progressive neurodegenerative disorder associated with aging, caused by an expanded polyglutamine (polyQ) repeat within the Huntingtin (HTT) protein. In HD, degeneration of the striatum and atrophy of the cortex are observed while cerebellum is less affected.

Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models / Franich, Nicholas R; Basso, Manuela; André, Emily A; Ochaba, Joseph; Kumar, Amit; Thein, Soe; Fote, Gianna; Kachemov, Marketta; Lau, Alice L; Yeung, Sylvia Y; Osmand, Alexander; Zeitlin, Scott O; Ratan, Rajiv R; Thompson, Leslie M; Steffan, Joan S. - In: JOURNAL OF HUNTINGTON’S DISEASE. - ISSN 1879-6397. - 7:2(2018), pp. 137-150-150. [10.3233/JHD-170274]

Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models

Basso, Manuela;
2018-01-01

Abstract

Huntington's disease (HD) is a progressive neurodegenerative disorder associated with aging, caused by an expanded polyglutamine (polyQ) repeat within the Huntingtin (HTT) protein. In HD, degeneration of the striatum and atrophy of the cortex are observed while cerebellum is less affected.
2018
2
Franich, Nicholas R; Basso, Manuela; André, Emily A; Ochaba, Joseph; Kumar, Amit; Thein, Soe; Fote, Gianna; Kachemov, Marketta; Lau, Alice L; Yeung, Sylvia Y; Osmand, Alexander; Zeitlin, Scott O; Ratan, Rajiv R; Thompson, Leslie M; Steffan, Joan S
Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models / Franich, Nicholas R; Basso, Manuela; André, Emily A; Ochaba, Joseph; Kumar, Amit; Thein, Soe; Fote, Gianna; Kachemov, Marketta; Lau, Alice L; Yeung, Sylvia Y; Osmand, Alexander; Zeitlin, Scott O; Ratan, Rajiv R; Thompson, Leslie M; Steffan, Joan S. - In: JOURNAL OF HUNTINGTON’S DISEASE. - ISSN 1879-6397. - 7:2(2018), pp. 137-150-150. [10.3233/JHD-170274]
File in questo prodotto:
File Dimensione Formato  
jhd-7-jhd170274.pdf

accesso aperto

Tipologia: Versione editoriale (Publisher’s layout)
Licenza: Creative commons
Dimensione 528.04 kB
Formato Adobe PDF
528.04 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11572/222708
Citazioni
  • ???jsp.display-item.citation.pmc??? 9
  • Scopus 13
  • ???jsp.display-item.citation.isi??? 11
social impact