期刊
JOURNAL OF BIOLOGICAL CHEMISTRY
卷 294, 期 18, 页码 7160-7168出版社
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.REV118.001188
关键词
neurodegeneration; protein aggregation; intrinsically disordered protein; Alzheimer disease; biomaterials; amyloid; Parkinson disease; amyotrophic lateral sclerosis (ALS) (Lou Gehrig disease); material properties; phase separation
资金
- NINDS NIH HHS [R00 NS096217] Funding Source: Medline
Phase separation of biomolecules leading to the formation of assemblies with distinct material properties has recently emerged as a new paradigm underlying subcellular organization. The discovery that disordered proteins, long associated with aggregation in neurodegenerative disease, are also implicated in driving liquid phase separation has galvanized significant interest in exploring the relationship between misregulated phase transitions and disease. This review summarizes recent work linking liquid phase separation to neurodegeneration, highlighting a pathological role for altered phase behavior and material properties of proteins assembled via liquid phase separation. The techniques that recent and current work in this area have deployed are also discussed, as is the potential for these discoveries to promote new research directions for investigating the molecular etiologies of neurodegenerative diseases.
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