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Reversal of Neurological Defects in a Mouse Model of Rett Syndrome
Oleh:
Guy, Jacky
;
Gan, Jian
;
Selfridge, Jim
;
Cobb, Stuart
;
Bird, Adrian
Jenis:
Article from Bulletin/Magazine
Dalam koleksi:
SCIENCE (keterangan: ada di Proquest) vol. 315 no. 5815 (Feb. 2007)
,
page 1143.
Topik:
Medicine
Ketersediaan
Perpustakaan FK
Nomor Panggil:
S01.K.2007.02
Non-tandon:
1 (dapat dipinjam: 0)
Tandon:
tidak ada
Lihat Detail Induk
Isi artikel
Rett syndrome is an autism spectrum disorder caused by mosaic expression of mutant copies of the X-linked MECP2 gene in neurons. However, neurons do not die, which suggests that this is not a neurodegenerative disorder. An important question for future therapeutic approaches to this and related disorders concerns phenotypic reversibility. Can viable but defective neurons be repaired, or is the damage done during development without normal MeCP2 irrevocable? Using a mouse model, we demonstrate robust phenotypic reversal, as activation of MeCP2 expression leads to striking loss of advan(ed neurological symptoms in both immature and mature adult animals.
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