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.| 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
