The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
Expansion of CAG trinucleotide repeat within the coding region of several genes results in
the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of …
the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of …
Localized and efficient curli nucleation requires the chaperone-like amyloid assembly protein CsgF
AA Nenninger, LS Robinson… - Proceedings of the …, 2009 - National Acad Sciences
Elucidation of the early events in amyloidogenesis is key to understanding the pathology of,
and developing therapies for, amyloid diseases. Critical informants about these early events …
and developing therapies for, amyloid diseases. Critical informants about these early events …
Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3
J Boy, T Schmidt, H Wolburg, A Mack… - Human molecular …, 2009 - academic.oup.com
Abstract Spinocerebellar ataxia type 3 (SCA3) is caused by the expansion of a CAG repeat
tract that affects the MJD1 gene which encodes the ataxin-3 protein. In order to analyze …
tract that affects the MJD1 gene which encodes the ataxin-3 protein. In order to analyze …
Virus-Induced Chaperone-Enriched (VICE) domains function as nuclear protein quality control centers during HSV-1 infection
Virus-Induced Chaperone-Enriched (VICE) domains form adjacent to nuclear viral
replication compartments (RC) during the early stages of HSV-1 infection. Between 2 and 3 …
replication compartments (RC) during the early stages of HSV-1 infection. Between 2 and 3 …
Identification and functional dissection of localization signals within ataxin-3
PMA Antony, S Mäntele, P Mollenkopf, J Boy… - Neurobiology of …, 2009 - Elsevier
Spinocerebellar ataxia type 3 (SCA3) or Machado–Joseph disease (MJD) belongs to a
group of autosomal dominant neurodegenerative diseases, which are caused by the …
group of autosomal dominant neurodegenerative diseases, which are caused by the …
Nucleocytoplasmic shuttling activity of ataxin-3
Spinocerebellar ataxia type-3, also known as Machado-Joseph Disease (MJD), is one of
many inherited neurodegenerative disorders caused by polyglutamine-encoding CAG …
many inherited neurodegenerative disorders caused by polyglutamine-encoding CAG …
Molecular pathogenesis of spinocerebellar ataxia type 1 disease
S Kang, S Hong - Molecules and cells, 2009 - Springer
Abstract Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative
disorder characterized by ataxia and progressive motor deterioration. SCA1 is associated …
disorder characterized by ataxia and progressive motor deterioration. SCA1 is associated …
Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk
JE Young, GA Garden, RA Martinez… - Journal of …, 2009 - Soc Neuroscience
Spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disorder caused
by a polyglutamine (polyQ) repeat expansion in the androgen receptor (AR). PolyQ-AR …
by a polyglutamine (polyQ) repeat expansion in the androgen receptor (AR). PolyQ-AR …
Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity
Background Misfolding-and aggregation-prone proteins underlying Parkinson's,
Huntington's and Machado-Joseph diseases, namely α-synuclein, huntingtin, and ataxin-3 …
Huntington's and Machado-Joseph diseases, namely α-synuclein, huntingtin, and ataxin-3 …
Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice
T Sato, M Miura, M Yamada, T Yoshida… - Human molecular …, 2009 - academic.oup.com
We herein provide a thorough description of new transgenic mouse models for dentatorubral–
pallidoluysian atrophy (DRPLA) harboring a single copy of the full-length human mutant …
pallidoluysian atrophy (DRPLA) harboring a single copy of the full-length human mutant …