Polyglutamine Solution-State Structural Propensity Is Repeat Length Dependent
نویسندگان
چکیده
منابع مشابه
Polyglutamine repeat length-dependent proteolysis of huntingtin.
Amino-terminal fragments of huntingtin, which contain the expanded polyglutamine repeat, have been proposed to contribute to the pathology of Huntington's disease (HD). Data supporting this claim have been generated from patients with HD in which truncated amino-terminal fragments forming intranuclear inclusions have been observed, and from animal and cell-based models of HD where it has been d...
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The dependence of disease risk and age-of-onset on expanded CAG repeat length in diseases like Huntington's disease (HD) is well established and correlates with the repeat-length-dependent nucleation kinetics of polyglutamine (polyGln) aggregation. The wide variation in ages of onset among patients with the same repeat length, however, suggests a role for modifying factors. Here we describe the...
متن کاملStructural characterization of polyglutamine fibrils by solid-state NMR spectroscopy.
Protein aggregation via polyglutamine stretches occurs in a number of severe neurodegenerative diseases such as Huntington's disease. We have investigated fibrillar aggregates of polyglutamine peptides below, at, and above the toxicity limit of around 37 glutamine residues using solid-state NMR and electron microscopy. Experimental data are consistent with a dry fibril core of at least 70-80 Å ...
متن کاملAndrogen receptor polyglutamine repeat length affects receptor activity and C2C12 cell development.
Testosterone (T) has an anabolic effect on skeletal muscle and is believed to exert its local effects via the androgen receptor (AR). The AR harbors a polymorphic stretch of glutamine repeats demonstrated to inversely affect receptor transcriptional activity in prostate and kidney cells. The effects of AR glutamine repeat length on skeletal muscle are unknown. In this study we examined the effe...
متن کاملAndrogen Receptor Polyglutamine Repeat Length Affects Receptor Activity and C2c12 Cell Myogenic Potential
Title of Document: ANDROGEN RECEPTOR POLYGLUTAMINE REPEAT LENGTH AFFECTS RECEPTOR ACTIVITY AND C2C12 CELL MYOGENIC POTENTIAL
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ژورنال
عنوان ژورنال: The Journal of Physical Chemistry B
سال: 2019
ISSN: 1520-6106,1520-5207
DOI: 10.1021/acs.jpcb.9b01433