期刊
JOURNAL OF BIOLOGICAL CHEMISTRY
卷 294, 期 18, 页码 7115-7127出版社
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.TM118.001192
关键词
subcellular organelle; organelle; amyotrophic lateral sclerosis (ALS) (Lou Gehrig disease); RNA binding protein; chaperone; Cajal body; disaggregase; liquid-liquid phase separation; nucleolus; stress granule
资金
- National Institutes of Health [R01GM099836, R21NS090205, R21NS102687]
- Muscular Dystrophy Association (MDA)
- Amyotrophic Lateral Sclerosis Association (ALSA)
- Target ALS
- Life Extension Foundation
- Sanofi
- Office of the Assistant Secretary of Defense for Health Affairs, through the Amyotrophic Lateral Sclerosis Research Program [W81XWH-17-1-0237]
- Robert Packard Center for ALS Research at Johns Hopkins University
Eukaryotic cells organize their intracellular components into organelles that can be membrane-bound or membraneless. A large number of membraneless organelles, including nucleoli, Cajal bodies, P-bodies, and stress granules, exist as liquid droplets within the cell and arise from the condensation of cellular material in a process termed liquid-liquid phase separation (LLPS). Beyond a mere organizational tool, concentrating cellular components into membraneless organelles tunes biochemical reactions and improves cellular fitness during stress. In this review, we provide an overview of the molecular underpinnings of the formation and regulation of these membraneless organelles. This molecular understanding explains emergent properties of these membraneless organelles and shines new light on neurodegenerative diseases, which may originate from disturbances in LLPS and membraneless organelles.
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