Journal
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
Volume 1865, Issue 6, Pages 1076-1087Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbadis.2019.03.009
Keywords
Lysosomes; Kinesin; Dynein; Retrograde; Anterograde; Lysosomal storage diseases
Funding
- FONDECYT [1161065, 1180900, 1150186]
- European Union's Horizon 2020 research and innovation programme (RISE) under the Marie Sklodowska-Curie grant [734825]
- Basal project [AFB170005]
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Lysosomes are dynamic organelles, which can fuse with a variety of targets and undergo constant regeneration. They can move along microtubules in a retrograde and anterograde fashion by using motor proteins, kinesin and dynein, being main players in extracellular secretion, intracellular components degradation and recycling. Moreover, lysosomes interact with other intracellular organelles to regulate their turnover, such as ER, mitochondria and peroxisomes. The correct localization of lysosomes is relevant in several physiological processes, including appropriate antigen presentation, neurotransmission and receptors modulation in neuronal synapsis, whereas hepatic lysosomes and autophagy are master regulators of nutrient homeostasis. Alterations in lysosome function due to mutation of genes encoding lysosomal proteins, soluble hydrolases as well as membrane proteins, lead to lysosomal storage diseases (LSDs). Lysosomes containing undegraded substrates are finally stacked and therefore miss positioned inside the cell, leading to lysosomal dysfunction, which impacts a wide range of cellular functions.
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