Engine breakdown of lysosomes and related organelles and the resulting physiology

TL;DR

This paper explores how lysosomes and related organelles move within cells and how disruptions in this process can lead to diseases.

Contribution

The paper provides a detailed analysis of the mechanisms and factors involved in lysosome transport and its failure in disease.

Findings

Lysosome transport depends on motor proteins and regulatory factors like Rab GTPases.

Defective transport due to mutations can cause skin, neurological, and immunological diseases.

The orchestration of transport involves microtubules, actin filaments, and membrane effectors.

Abstract

Late endosomes/lysosomes (LE/Lys) and lysosome related organelles (LROs) move dynamically through cells which involves many levels of regulation. To reach their destination, they need to connect to the motor proteins dynein-dynactin, kinesin or myosin for long-range bidirectional transport along microtubules and short-range movement along actin filaments. This connection depends on various factors at the microtubule, including the MAP- and tubulin-code, as well as adaptors, Rab GTPases and effector proteins marking the LE/Lys and LRO membranes. Mutations affecting this transport results in defective LE/Lys or LRO cargo delivery often resulting in skin, neurological and/or immunological diseases. How LE/Lys and LRO transport is orchestrated and how it fails in disease states, will be discussed.

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