Universität zu Köln

Tadepalle, Nimesha and Rugarli, Elena, I (2021). Lipid Droplets in the Pathogenesis of Hereditary Spastic Paraplegia. Front. Mol. Biosci., 8. LAUSANNE: FRONTIERS MEDIA SA. ISSN 2296-889X

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Abstract

Hereditary spastic paraplegias (HSPs) are genetically heterogeneous conditions caused by the progressive dying back of the longest axons in the central nervous system, the corticospinal axons. A wealth of data in the last decade has unraveled disturbances of lipid droplet (LD) biogenesis, maturation, turnover and contact sites in cellular and animal models with perturbed expression and function of HSP proteins. As ubiquitous organelles that segregate neutral lipid into a phospholipid monolayer, LDs are at the cross-road of several processes including lipid metabolism and trafficking, energy homeostasis, and stress signaling cascades. However, their role in brain cells, especially in neurons remains enigmatic. Here, we review experimental findings linking LD abnormalities to defective function of proteins encoded by HSP genes, and discuss arising questions in the context of the pathogenesis of HSP.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Tadepalle, Nimesha UNSPECIFIED UNSPECIFIED UNSPECIFIED Rugarli, Elena, I UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-581147
DOI:	10.3389/fmolb.2021.673977
Journal or Publication Title:	Front. Mol. Biosci.
Volume:	8
Date:	2021
Publisher:	FRONTIERS MEDIA SA
Place of Publication:	LAUSANNE
ISSN:	2296-889X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SPINAL MUSCULAR-ATROPHY; MOTOR-NEURON DISEASE; ENDOPLASMIC-RETICULUM; ATLASTIN GTPASES; TROYER-SYNDROME; QUALITY-CONTROL; HUMAN SEIPIN; MICE REVEAL; PROTEIN; ER Multiple languages Biochemistry & Molecular Biology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/58114