Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia

Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia

Abstract Hereditary spastic paraplegias (HSPs) are caused by a length-dependent axonopathy of long corticospinal neurons, but how axons of these cortical projection neurons (PNs) degenerate remains elusive. We generated isogenic human pluripotent stem cell (hPSC) lines for two ATL1 missense mutation...

Full description

Bibliographic Details
Main Authors:	Yongchao Mou, Yi Dong, Zhenyu Chen, Kyle R. Denton, Michael O. Duff, Craig Blackstone, Su-Chun Zhang, Xue-Jun Li
Format:	Article
Language:	English
Published:	BMC 2020-12-01
Series:	Acta Neuropathologica Communications
Subjects:	Hereditary spastic paraplegia Human pluripotent stem cells Cortical projection neurons Cholesterol homeostasis Axonal degeneration Astrocytes
Online Access:	https://doi.org/10.1186/s40478-020-01088-0

Similar Items

Rescue axonal defects by targeting mitochondrial dynamics in hereditary spastic paraplegias
by: Yongchao Mou, et al.
Published: (2019-01-01)

Editorial: Hereditary Spastic Paraplegias: At the Crossroads of Molecular Pathways and Clinical Options
by: Andrea Martinuzzi, et al.
Published: (2021-06-01)

Clinical Spectrum of Hereditary Spastic Paraplegia in Children : A study of 74 cases
by: Roshan Koul, et al.
Published: (2013-08-01)

Repetitive Transcranial Magnetic Stimulation in Patients with Hereditary Spastic Paraplegia
by: Mehmet Ağırman, et al.
Published: (2011-06-01)

Repetitive Transcranial Magnetic Stimulation in Patients with Hereditary Spastic Paraplegia
by: Mehmet Ağırman, et al.
Published: (2011-06-01)

Clinical features of pure hereditary spastic paraplegia
by: Qian-qian WEI, et al.
Published: (2013-07-01)

Oxidative Stress-Induced Axon Fragmentation Is a Consequence of Reduced Axonal Transport in Hereditary Spastic Paraplegia SPAST Patient Neurons
by: Gautam Wali, et al.
Published: (2020-05-01)

Clinico-investigative profile of hereditary spastic paraplegia in children
by: Mahesh Kamate, et al.
Published: (2019-01-01)

Modeling of axonal endoplasmic reticulum network by spastic paraplegia proteins
by: Belgin Yalçın, et al.
Published: (2017-07-01)

Mutation in KIF5A c.610C>T Causing Hereditary Spastic Paraplegia with Axonal Sensorimotor Neuropathy
by: Mathieu Cuchanski, et al.
Published: (2018-07-01)

Neuroimaging in Hereditary Spastic Paraplegias: Current Use and Future Perspectives
by: Felipe Franco da Graça, et al.
Published: (2019-01-01)

Multiparametric 3T MRI evaluation of hereditary spastic paraplegia: A case report
by: Sonam Priya, et al.
Published: (2016-07-01)

Lipid Droplets in the Pathogenesis of Hereditary Spastic Paraplegia
by: Nimesha Tadepalle, et al.
Published: (2021-05-01)

Increased baroreceptor sensitivity in a patient with hereditary spastic paraplegia – type SPG11
by: Fischer Gerald, et al.
Published: (2018-09-01)

Efficacy of a Combined Treatment of Botulinum Toxin and Intensive Physiotherapy in Hereditary Spastic Paraplegia
by: Gabriella Paparella, et al.
Published: (2020-02-01)

Two novel homozygous mutations of CAPN1 in Chinese patients with hereditary spastic paraplegia and literatures review
by: Fang Peng, et al.
Published: (2019-04-01)

Going Too Far Is the Same as Falling Short†: Kinesin-3 Family Members in Hereditary Spastic Paraplegia
by: Dominik R. Gabrych, et al.
Published: (2019-09-01)

Pallidopyramidal Syndrome and Hereditary Spastic Paraplegia common features and diagnostic approach and therapeutic considerations
by: BULBOACA Corneliu Angelo,, et al.
Published: (2019-09-01)

Are Cognitive Changes in Hereditary Spastic Paraplegias Restricted to Complicated Forms?
by: Laís Alves Jacinto-Scudeiro, et al.
Published: (2019-05-01)

Hereditary spastic paraplegia associated with a rare IFIH1 mutation: a case report and literature review
by: Nan Liu, et al.
Published: (2019-08-01)

Novel Type of Complicated Autosomal Dominant Hereditary Spastic Paraplegia Associated with Congenital Distal Arthrogryposis Type I
by: Peter Hedera, et al.
Published: (2018-07-01)

Potential markers for sample size estimations in hereditary spastic paraplegia type 5
by: Qianqian Lin, et al.
Published: (2021-09-01)

Desflurane for management of decompressive laminectomy in a patient with hereditary spastic paraplegia: a case report
by: Masahiro Tada, et al.
Published: (2019-04-01)

Swimming in Deep Water: Zebrafish Modeling of Complicated Forms of Hereditary Spastic Paraplegia and Spastic Ataxia
by: Valentina Naef, et al.
Published: (2019-12-01)

Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands
by: Bas J. H. van Lith, et al.
Published: (2020-03-01)

Retinal nerve fibre layer loss in hereditary spastic paraplegias is restricted to complex phenotypes
by: Wiethoff Sarah, et al.
Published: (2012-11-01)

Corticospinal tract damage in HHH syndrome: a metabolic cause of hereditary spastic paraplegia
by: Giorgia Olivieri, et al.
Published: (2019-08-01)

Clinical spectrum and genetic landscape for hereditary spastic paraplegias in China
by: En-Lin Dong, et al.
Published: (2018-07-01)

Hereditary spastic paraplegia associated with thin corpus callosum Paraplegia espástica hereditária associada a hipoplasia de corpo caloso
by: Hélio A. Ghizoni Teive, et al.
Published: (2001-09-01)

Case series of Familial (Hereditary) Spastic Paraplegia
by: P. Yazdanpanah, M.D. +
Published: (2007-01-01)

Coexistence of Hereditary Spastic Paraplegia Type 4 and Narcolepsy: A Case Report
by: Takahiro Nagai, et al.
Published: (2021-02-01)

Hereditary Spastic Paraparesis with Thin Corpus Callosum: Characteristic MRI Findings
by: Eser S Sanverdi, et al.
Published: (2011-12-01)

Hereditary Spastic Paraparesis with Thin Corpus Callosum: Characteristic MRI Findings
by: Eser S Sanverdi, et al.
Published: (2011-12-01)

Functional effects of botulinum toxin type A in the hip adductors and subsequent stretching in patients with hereditary spastic paraplegia
by: Bas J.H. van Lith, et al.
Published: (2019-04-01)

Novel Mutations in Endoplasmic Reticulum Lipid Raft-associated Protein 2 Gene Cause Pure Hereditary Spastic Paraplegia Type 18
by: Wo-Tu Tian, et al.
Published: (2016-01-01)

Motor Evoked Potentials in Hereditary Spastic Paraplegia—A Systematic Review
by: Sue-Faye Siow, et al.
Published: (2019-09-01)

Improving gait adaptability in patients with hereditary spastic paraplegia (Move-HSP): study protocol for a randomized controlled trial
by: Lotte van de Venis, et al.
Published: (2021-01-01)

Mitochondrial Function in Hereditary Spastic Paraplegia: Deficits in SPG7 but Not SPAST Patient-Derived Stem Cells
by: Gautam Wali, et al.
Published: (2020-08-01)

Chinese families with autosomal recessive hereditary spastic paraplegia caused by mutations in SPG11
by: Xueping Chen, et al.
Published: (2020-01-01)

Naringenin Ameliorates Drosophila ReepA Hereditary Spastic Paraplegia-Linked Phenotypes
by: Barbara Napoli, et al.
Published: (2019-11-01)