ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia

The endoplasmic reticulum (ER) is the most abundant and widespread organelle in cells. Its peculiar membrane architecture, formed by an intricate network of tubules and cisternae, is critical to its multifaceted function. Regulation of ER morphology is coordinated by a few ER-specific membrane prote...

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Autores principales:	Sonia Sonda, Diana Pendin, Andrea Daga
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	endoplasmic reticulum hereditary spastic paraplegia ER-shaping proteins Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/8fd07e3868d443a29590540751b68264
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spelling	oai:doaj.org-article:8fd07e3868d443a29590540751b682642021-11-25T17:08:19ZER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia10.3390/cells101128702073-4409https://doaj.org/article/8fd07e3868d443a29590540751b682642021-10-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/2870https://doaj.org/toc/2073-4409The endoplasmic reticulum (ER) is the most abundant and widespread organelle in cells. Its peculiar membrane architecture, formed by an intricate network of tubules and cisternae, is critical to its multifaceted function. Regulation of ER morphology is coordinated by a few ER-specific membrane proteins and is thought to be particularly important in neurons, where organized ER membranes are found even in the most distant neurite terminals. Mutation of ER-shaping proteins has been implicated in the neurodegenerative disease hereditary spastic paraplegia (HSP). In this review we discuss the involvement of these proteins in the pathogenesis of HSP, focusing on the experimental evidence linking their molecular function to disease onset. Although the precise biochemical activity of some ER-related HSP proteins has been elucidated, the pathological mechanism underlying ER-linked HSP is still undetermined and needs to be further investigated.Sonia SondaDiana PendinAndrea DagaMDPI AGarticleendoplasmic reticulumhereditary spastic paraplegiaER-shaping proteinsBiology (General)QH301-705.5ENCells, Vol 10, Iss 2870, p 2870 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	endoplasmic reticulum hereditary spastic paraplegia ER-shaping proteins Biology (General) QH301-705.5
spellingShingle	endoplasmic reticulum hereditary spastic paraplegia ER-shaping proteins Biology (General) QH301-705.5 Sonia Sonda Diana Pendin Andrea Daga ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia
description	The endoplasmic reticulum (ER) is the most abundant and widespread organelle in cells. Its peculiar membrane architecture, formed by an intricate network of tubules and cisternae, is critical to its multifaceted function. Regulation of ER morphology is coordinated by a few ER-specific membrane proteins and is thought to be particularly important in neurons, where organized ER membranes are found even in the most distant neurite terminals. Mutation of ER-shaping proteins has been implicated in the neurodegenerative disease hereditary spastic paraplegia (HSP). In this review we discuss the involvement of these proteins in the pathogenesis of HSP, focusing on the experimental evidence linking their molecular function to disease onset. Although the precise biochemical activity of some ER-related HSP proteins has been elucidated, the pathological mechanism underlying ER-linked HSP is still undetermined and needs to be further investigated.
format	article
author	Sonia Sonda Diana Pendin Andrea Daga
author_facet	Sonia Sonda Diana Pendin Andrea Daga
author_sort	Sonia Sonda
title	ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia
title_short	ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia
title_full	ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia
title_fullStr	ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia
title_full_unstemmed	ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia
title_sort	er morphology in the pathogenesis of hereditary spastic paraplegia
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/8fd07e3868d443a29590540751b68264
work_keys_str_mv	AT soniasonda ermorphologyinthepathogenesisofhereditaryspasticparaplegia AT dianapendin ermorphologyinthepathogenesisofhereditaryspasticparaplegia AT andreadaga ermorphologyinthepathogenesisofhereditaryspasticparaplegia
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ER Morphology in the Pathogenesis of Hereditary Spastic Paraplegia

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