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1 f the gene products (huntingtin, atrophin-1 (DRPLA), ataxin-3, and androgen receptor) associated with
9 JD), and dentatorubropallidoluysian atrophy (DRPLA) in patients with sporadic and inherited ataxia.
11 (HD) and dentatorubropallidoluysian atrophy (DRPLA) using 31P magnetic resonance spectroscopy (MRS).
12 Dentato-rubral and pallido-luysian atrophy (DRPLA) is one of the family of neurodegenerative disease
14 rative dentatorubral-pallidoluysian atrophy (DRPLA) and that is essential for development of multiple
15 ), and dentatorubral-pallidoluysian atrophy (DRPLA) are produced by genes containing a variably incre
16 Dentatorubral and pallidoluysian atrophy (DRPLA) is a member of a family of progressive neurodegen
17 Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disord
18 SCA1), dentatorubral pallidoluysian atrophy (DRPLA) Machado-Joseph disease (MJD or SCA3) and SCA2.
19 ), DentatoRubral and PallidoLuysian Atrophy (DRPLA), and Spino-Cerebellar Ataxia (SCA), have been con
20 isease dentatorubral-pallidoluysian atrophy (DRPLA), progressive acquirement of an ataxic phenotype i
21 (HD), dentatorubral-pallidoluysian atrophy (DRPLA), spinocerebellar ataxia type 1 (SCA1), Machado-Jo
22 or exception is the cerebellum, which in HD, DRPLA, SCA1 and MJD has a smaller repeat relative to the
23 lude that the evolution of neuropathology in DRPLA involves proteolytic processing of mutant atrophin
24 similar in size to the truncation product in DRPLA patient tissue, showed increased nuclear labeling,
25 find that canonical autophagy is stalled in DRPLA mice and in human fibroblasts from patients of DRP
26 gy blockage in several conditions, including DRPLA and Vici syndrome, an early-onset autolysosomal pa
29 uronal nuclei in a transgenic mouse model of DRPLA, and may underlie the disease phenotype.Using the
33 presymptomatic patients) and 2 gene-positive DRPLA patients (1 presymptomatic patient) were studied.
34 and fetal tissues, is highly related to the DRPLA gene, in which expansion of a CAG triplet appears
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