PMID- 35227276
OWN - NLM
STAT- MEDLINE
DCOM- 20220316
LR  - 20231102
IS  - 2045-8118 (Electronic)
IS  - 2045-8118 (Linking)
VI  - 19
IP  - 1
DP  - 2022 Feb 28
TI  - The neurovascular unit in leukodystrophies: towards solving the puzzle.
PG  - 18
LID - 10.1186/s12987-022-00316-0 [doi]
LID - 18
AB  - The neurovascular unit (NVU) is a highly organized multicellular system localized 
      in the brain, formed by neuronal, glial (astrocytes, oligodendrocytes, and 
      microglia) and vascular (endothelial cells and pericytes) cells. The blood-brain 
      barrier, a complex and dynamic endothelial cell barrier in the brain 
      microvasculature that separates the blood from the brain parenchyma, is a 
      component of the NVU. In a variety of neurological disorders, including 
      Alzheimer's disease, multiple sclerosis, and stroke, dysfunctions of the NVU 
      occurs. There is, however, a lack of knowledge regarding the NVU function in 
      leukodystrophies, which are rare monogenic disorders that primarily affect the 
      white matter. Since leukodystrophies are rare diseases, human brain tissue 
      availability is scarce and representative animal models that significantly 
      recapitulate the disease are difficult to develop. The introduction of human 
      induced pluripotent stem cells (hiPSC) now makes it possible to surpass these 
      limitations while maintaining the ability to work in a biologically relevant 
      human context and safeguarding the genetic background of the patient. This review 
      aims to provide further insights into the NVU functioning in leukodystrophies, 
      with a special focus on iPSC-derived models that can be used to dissect 
      neurovascular pathophysiology in these diseases.
CI  - (c) 2022. The Author(s).
FAU - Zarekiani, Parand
AU  - Zarekiani P
AD  - Department of Pathology, Amsterdam Neuroscience, Amsterdam UMC, Vrije 
      Universiteit Amsterdam, de Boelelaan 1117, Amsterdam, The Netherlands.
AD  - Amsterdam Leukodystrophy Center, Amsterdam UMC, Amsterdam, The Netherlands.
AD  - Department of Molecular Cell Biology and Immunology, Amsterdam Neuroscience, 
      Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, The 
      Netherlands.
FAU - Nogueira Pinto, Henrique
AU  - Nogueira Pinto H
AD  - Department of Molecular Cell Biology and Immunology, Amsterdam Neuroscience, 
      Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, The 
      Netherlands.
AD  - Department of Translational Neuroscience, University Medical Center Utrecht Brain 
      Center, Utrecht University, Utrecht, The Netherlands.
FAU - Hol, Elly M
AU  - Hol EM
AD  - Department of Translational Neuroscience, University Medical Center Utrecht Brain 
      Center, Utrecht University, Utrecht, The Netherlands.
FAU - Bugiani, Marianna
AU  - Bugiani M
AD  - Department of Pathology, Amsterdam Neuroscience, Amsterdam UMC, Vrije 
      Universiteit Amsterdam, de Boelelaan 1117, Amsterdam, The Netherlands.
AD  - Amsterdam Leukodystrophy Center, Amsterdam UMC, Amsterdam, The Netherlands.
FAU - de Vries, Helga E
AU  - de Vries HE
AD  - Department of Molecular Cell Biology and Immunology, Amsterdam Neuroscience, 
      Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, The 
      Netherlands. he.devries@amsterdamumc.nl.
LA  - eng
GR  - VENI 016.196.107/ZONMW_/ZonMw/Netherlands
GR  - HMM2.0 CONNECT 18957/Nederlandse Organisatie voor Wetenschappelijk Onderzoek/
PT  - Journal Article
PT  - Review
DEP - 20220228
PL  - England
TA  - Fluids Barriers CNS
JT  - Fluids and barriers of the CNS
JID - 101553157
SB  - IM
MH  - Animals
MH  - Astrocytes
MH  - Blood-Brain Barrier
MH  - Endothelial Cells
MH  - Humans
MH  - *Induced Pluripotent Stem Cells
MH  - *Neurodegenerative Diseases
PMC - PMC8887016
OTO - NOTNLM
OT  - Astrocyte
OT  - Blood-brain barrier
OT  - Endothelium
OT  - In vitro models
OT  - Induced pluripotent stem cells
OT  - Leukodystrophies
OT  - Microglia
OT  - Neurovascular unit
OT  - Pericyte
COIS- The authors declare that they have no competing interests.
EDAT- 2022/03/02 06:00
MHDA- 2022/03/17 06:00
PMCR- 2022/02/28
CRDT- 2022/03/01 05:42
PHST- 2021/12/22 00:00 [received]
PHST- 2022/02/11 00:00 [accepted]
PHST- 2022/03/01 05:42 [entrez]
PHST- 2022/03/02 06:00 [pubmed]
PHST- 2022/03/17 06:00 [medline]
PHST- 2022/02/28 00:00 [pmc-release]
AID - 10.1186/s12987-022-00316-0 [pii]
AID - 316 [pii]
AID - 10.1186/s12987-022-00316-0 [doi]
PST - epublish
SO  - Fluids Barriers CNS. 2022 Feb 28;19(1):18. doi: 10.1186/s12987-022-00316-0.