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Golgi trafficking defects in postnatal microcephaly: The evidence for "Golgipathies"

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Golgi trafficking defects in postnatal microcephaly : The evidence for "Golgipathies". / Passemard, Sandrine; Perez, Franck; Colin-Lemesre, Emilie; Rasika, Sowmyalakshmi; Gressens, Pierre; El Ghouzzi, Vincent.

In: Progress in Neurobiology, Vol. 153, 06.2017, p. 46-63.

Research output: Contribution to journalArticle

Harvard

Passemard, S, Perez, F, Colin-Lemesre, E, Rasika, S, Gressens, P & El Ghouzzi, V 2017, 'Golgi trafficking defects in postnatal microcephaly: The evidence for "Golgipathies"', Progress in Neurobiology, vol. 153, pp. 46-63. https://doi.org/10.1016/j.pneurobio.2017.03.007

APA

Passemard, S., Perez, F., Colin-Lemesre, E., Rasika, S., Gressens, P., & El Ghouzzi, V. (2017). Golgi trafficking defects in postnatal microcephaly: The evidence for "Golgipathies". Progress in Neurobiology, 153, 46-63. https://doi.org/10.1016/j.pneurobio.2017.03.007

Vancouver

Passemard S, Perez F, Colin-Lemesre E, Rasika S, Gressens P, El Ghouzzi V. Golgi trafficking defects in postnatal microcephaly: The evidence for "Golgipathies". Progress in Neurobiology. 2017 Jun;153:46-63. https://doi.org/10.1016/j.pneurobio.2017.03.007

Author

Passemard, Sandrine ; Perez, Franck ; Colin-Lemesre, Emilie ; Rasika, Sowmyalakshmi ; Gressens, Pierre ; El Ghouzzi, Vincent. / Golgi trafficking defects in postnatal microcephaly : The evidence for "Golgipathies". In: Progress in Neurobiology. 2017 ; Vol. 153. pp. 46-63.

Bibtex Download

@article{2240dd0d622847b096b8885972dac19c,
title = "Golgi trafficking defects in postnatal microcephaly: The evidence for {"}Golgipathies{"}",
abstract = "The Golgi apparatus plays a central role in cell homeostasis, not only in processing and maturing newly synthesized proteins and lipids but also in orchestrating their sorting, packing, routing and recycling on the way to their final destination. These multiple secretory pathways require a complex ballet of vesicular and tubular carriers that continuously bud off from donor membranes and fuse to acceptor membranes. Membrane trafficking is particularly prominent in axons, where cargo molecules have a long way to travel before they reach the synapse, and in oligodendrocytes, which require an immense increase in membrane surface in order to sheathe axons in myelin. Interestingly, in recent years, genes encoding Golgi-associated proteins with a role in membrane trafficking have been found to be defective in an increasing number of inherited disorders whose clinical manifestations include postnatal-onset microcephaly (POM), white matter defects and intellectual disability. Several of these genes encode RAB GTPases, RAB-effectors or RAB-regulating proteins, linking POM and intellectual disability to RAB-dependent Golgi trafficking pathways and suggesting that their regulation is critical to postnatal brain maturation and function. Here, we review the key roles of the Golgi apparatus in post-mitotic neurons and the oligodendrocytes that myelinate them, and provide an overview of these Golgi-associated POM-causing genes, their function in Golgi organization and trafficking and the likely mechanisms that may link dysfunctions in RAB-dependent regulatory pathways with POM.",
keywords = "Journal Article, Review",
author = "Sandrine Passemard and Franck Perez and Emilie Colin-Lemesre and Sowmyalakshmi Rasika and Pierre Gressens and {El Ghouzzi}, Vincent",
note = "Copyright {\circledC} 2017 Elsevier Ltd. All rights reserved.",
year = "2017",
month = "6",
doi = "10.1016/j.pneurobio.2017.03.007",
language = "English",
volume = "153",
pages = "46--63",
journal = "Progress in Neurobiology",
issn = "0301-0082",
publisher = "Elsevier Limited",

}

RIS (suitable for import to EndNote) Download

TY - JOUR

T1 - Golgi trafficking defects in postnatal microcephaly

T2 - The evidence for "Golgipathies"

AU - Passemard, Sandrine

AU - Perez, Franck

AU - Colin-Lemesre, Emilie

AU - Rasika, Sowmyalakshmi

AU - Gressens, Pierre

AU - El Ghouzzi, Vincent

N1 - Copyright © 2017 Elsevier Ltd. All rights reserved.

PY - 2017/6

Y1 - 2017/6

N2 - The Golgi apparatus plays a central role in cell homeostasis, not only in processing and maturing newly synthesized proteins and lipids but also in orchestrating their sorting, packing, routing and recycling on the way to their final destination. These multiple secretory pathways require a complex ballet of vesicular and tubular carriers that continuously bud off from donor membranes and fuse to acceptor membranes. Membrane trafficking is particularly prominent in axons, where cargo molecules have a long way to travel before they reach the synapse, and in oligodendrocytes, which require an immense increase in membrane surface in order to sheathe axons in myelin. Interestingly, in recent years, genes encoding Golgi-associated proteins with a role in membrane trafficking have been found to be defective in an increasing number of inherited disorders whose clinical manifestations include postnatal-onset microcephaly (POM), white matter defects and intellectual disability. Several of these genes encode RAB GTPases, RAB-effectors or RAB-regulating proteins, linking POM and intellectual disability to RAB-dependent Golgi trafficking pathways and suggesting that their regulation is critical to postnatal brain maturation and function. Here, we review the key roles of the Golgi apparatus in post-mitotic neurons and the oligodendrocytes that myelinate them, and provide an overview of these Golgi-associated POM-causing genes, their function in Golgi organization and trafficking and the likely mechanisms that may link dysfunctions in RAB-dependent regulatory pathways with POM.

AB - The Golgi apparatus plays a central role in cell homeostasis, not only in processing and maturing newly synthesized proteins and lipids but also in orchestrating their sorting, packing, routing and recycling on the way to their final destination. These multiple secretory pathways require a complex ballet of vesicular and tubular carriers that continuously bud off from donor membranes and fuse to acceptor membranes. Membrane trafficking is particularly prominent in axons, where cargo molecules have a long way to travel before they reach the synapse, and in oligodendrocytes, which require an immense increase in membrane surface in order to sheathe axons in myelin. Interestingly, in recent years, genes encoding Golgi-associated proteins with a role in membrane trafficking have been found to be defective in an increasing number of inherited disorders whose clinical manifestations include postnatal-onset microcephaly (POM), white matter defects and intellectual disability. Several of these genes encode RAB GTPases, RAB-effectors or RAB-regulating proteins, linking POM and intellectual disability to RAB-dependent Golgi trafficking pathways and suggesting that their regulation is critical to postnatal brain maturation and function. Here, we review the key roles of the Golgi apparatus in post-mitotic neurons and the oligodendrocytes that myelinate them, and provide an overview of these Golgi-associated POM-causing genes, their function in Golgi organization and trafficking and the likely mechanisms that may link dysfunctions in RAB-dependent regulatory pathways with POM.

KW - Journal Article

KW - Review

U2 - 10.1016/j.pneurobio.2017.03.007

DO - 10.1016/j.pneurobio.2017.03.007

M3 - Article

C2 - 28377289

VL - 153

SP - 46

EP - 63

JO - Progress in Neurobiology

JF - Progress in Neurobiology

SN - 0301-0082

ER -

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