Lysosome motility and distribution: Relevance in health and disease

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

卷:1865

Review

Esteban Oyarzun, Juan¹  Lagos, Jonathan^1,2  Carmen Vazquez, Mary³  Valls, Cristian^4,5  De la Fuente, Catalina^4,5  Isabel Yuseff, Maria²  Alvarez, Alejandra R.^4,5  Zanlungo, Silvana¹ 

[1] Pontificia Univ Catolica Chile, Dept Gastroenterol, Fac Med, Santiago, Chile

[2] Pontificia Univ Catolica Chile, Fac Biol Sci, Dept Cellular & Mol Biol, Lab Immune Cell Biol, Santiago, Chile

[3] Univ Andres Bello, Fac Biol Sci, Dept Biol Sci, Santiago, Chile

[4] Pontificia Univ Catolica Chile, Biol Sci Fac, Lab Cell Signaling, Dept Cell & Mol Biol, Santiago, Chile

[5] Pontificia Univ Catolica Chile, CARE UC, Santiago, Chile

关键词: Lysosomes;    Kinesin;    Dynein;    Retrograde;    Anterograde;    Lysosomal storage diseases;   

DOI  :  10.1016/j.bbadis.2019.03.009

来源: Elsevier

【 摘 要 】

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.

【 授权许可】

Free