The neurovascular unit in leukodystrophies: towards solving the puzzle.
Astrocyte
Blood–brain barrier
Endothelium
In vitro models
Induced pluripotent stem cells
Leukodystrophies
Microglia
Neurovascular unit
Pericyte
Journal
Fluids and barriers of the CNS
ISSN: 2045-8118
Titre abrégé: Fluids Barriers CNS
Pays: England
ID NLM: 101553157
Informations de publication
Date de publication:
28 Feb 2022
28 Feb 2022
Historique:
received:
22
12
2021
accepted:
11
02
2022
entrez:
1
3
2022
pubmed:
2
3
2022
medline:
17
3
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 35227276
doi: 10.1186/s12987-022-00316-0
pii: 10.1186/s12987-022-00316-0
pmc: PMC8887016
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
18Subventions
Organisme : ZonMw
ID : VENI 016.196.107
Pays : Netherlands
Organisme : Nederlandse Organisatie voor Wetenschappelijk Onderzoek
ID : HMM2.0 CONNECT 18957
Informations de copyright
© 2022. The Author(s).
Références
Glia. 2003 Dec;44(3):283-95
pubmed: 14603469
Immunity. 2016 Mar 15;44(3):439-449
pubmed: 26982352
Acta Neuropathol. 2010 Jan;119(1):7-35
pubmed: 20012068
Acta Neuropathol. 2022 Feb;143(2):125-141
pubmed: 34878590
Ann Neurol. 1984 Jan;15(1):49-54
pubmed: 6712192
Dev Med Child Neurol. 2013 Jan;55(1):46-57
pubmed: 23121296
J Neurosci Methods. 2009 Jun 15;180(2):261-72
pubmed: 19439360
J Immunol. 1998 Nov 1;161(9):5016-26
pubmed: 9794439
J Neurol Sci. 2013 Dec 15;335(1-2):75-81
pubmed: 24139698
Proc Natl Acad Sci U S A. 1998 Feb 17;95(4):1840-5
pubmed: 9465104
Neuromolecular Med. 2003;4(1-2):73-94
pubmed: 14528054
Nat Neurosci. 2015 Jul;18(7):942-52
pubmed: 26108722
Neuropathol Appl Neurobiol. 2011 Feb;37(1):94-113
pubmed: 21062344
Hypertension. 2003 Jun;41(6):1253-8
pubmed: 12743011
Proc Natl Acad Sci U S A. 2001 Nov 20;98(24):14108-13
pubmed: 11717465
Nat Commun. 2021 Aug 5;12(1):4730
pubmed: 34354063
Front Cell Neurosci. 2019 Jun 06;13:230
pubmed: 31244605
Neurology. 2001 Sep 25;57(6):1066-70
pubmed: 11571335
Acta Neuropathol. 2022 Feb;143(2):159-177
pubmed: 34878591
Proc Natl Acad Sci U S A. 2012 Jan 24;109(4):1317-22
pubmed: 22232668
Neuron. 2002 Nov 14;36(4):585-96
pubmed: 12441049
Neuron. 2019 Mar 6;101(5):920-937.e13
pubmed: 30685224
Neurology. 2011 Sep 27;77(13):1287-94
pubmed: 21917775
Drug Metab Dispos. 2009 Mar;37(3):635-43
pubmed: 19047468
Nat Rev Mol Cell Biol. 2004 Sep;5(9):687-98
pubmed: 15340377
Cell Transplant. 2013;22(9):1541-52
pubmed: 23067568
Brain Pathol. 2018 Nov;28(6):832-843
pubmed: 29757481
Cell. 2006 Aug 25;126(4):663-76
pubmed: 16904174
Neurology. 2016 Oct 25;87(17):1777-1786
pubmed: 27664989
Front Immunol. 2019 Sep 20;10:2199
pubmed: 31616414
Hum Mol Genet. 2004 Nov 1;13(21):2581-94
pubmed: 15367490
Bioessays. 2004 Mar;26(3):225-34
pubmed: 14988924
Elife. 2018 Mar 21;7:
pubmed: 29561727
AJNR Am J Neuroradiol. 2001 Mar;22(3):541-52
pubmed: 11237983
J Neurosci Methods. 2008 Sep 15;174(1):31-41
pubmed: 18672002
Neurobiol Aging. 2014 Mar;35(3):565-75
pubmed: 24138987
Nat Protoc. 2014 Oct;9(10):2329-40
pubmed: 25188634
Lancet Neurol. 2012 Nov;11(11):973-85
pubmed: 23079554
Neurol Sci. 2016 Sep;37(9):1565-9
pubmed: 27338940
Hypertension. 2002 May;39(5):976-81
pubmed: 12019279
Lab Chip. 2022 Apr 12;22(8):1615-1629
pubmed: 35333271
Eur J Neurosci. 2011 Jun;33(12):2179-86
pubmed: 21501259
J Med Genet. 1995 Nov;32(11):881-4
pubmed: 8592332
Vasc Cell. 2013 Feb 19;5(1):4
pubmed: 23422217
Trends Biotechnol. 2018 Apr;36(4):457-472
pubmed: 29422410
Acta Neuropathol. 2009 Oct;118(4):531-9
pubmed: 19562355
Nature. 1996 Oct 24;383(6602):707-10
pubmed: 8878478
Neuroscience. 2010 Jul 28;168(4):941-56
pubmed: 19761815
Nat Rev Neurosci. 2013 Apr;14(4):265-77
pubmed: 23481483
J Cereb Blood Flow Metab. 2016 Feb;36(2):302-25
pubmed: 26661176
J Chem Neuroanat. 2018 Oct;92:48-60
pubmed: 29885791
Cell Mol Life Sci. 1999 Dec;56(11-12):894-907
pubmed: 11212324
Neuroreport. 2018 May 2;29(7):588-593
pubmed: 29570159
EMBO Mol Med. 2021 Feb 5;13(2):e12889
pubmed: 33350588
N Engl J Med. 1991 Jan 3;324(1):18-22
pubmed: 1670590
Nature. 2006 Oct 12;443(7112):700-4
pubmed: 17036005
Brain Pathol. 2014 Sep;24(5):495-509
pubmed: 25323665
Neuropediatrics. 1990 Aug;21(3):164-8
pubmed: 2234324
Nat Methods. 2019 Nov;16(11):1169-1175
pubmed: 31591580
Cardiovasc Res. 2013 Jul 15;99(2):251-9
pubmed: 23519267
Nature. 2010 Nov 11;468(7321):232-43
pubmed: 21068832
Mol Neurobiol. 2019 Dec;56(12):8237-8254
pubmed: 31209783
Genes Dis. 2017 Dec;4(4):185-195
pubmed: 29911158
Ann Neurol. 2015 Jan;77(1):114-31
pubmed: 25382142
Handb Clin Neurol. 2018;148:693-700
pubmed: 29478608
Acta Neuropathol Commun. 2021 Jun 3;9(1):103
pubmed: 34082828
Trends Immunol. 2012 Dec;33(12):579-89
pubmed: 22926201
Acta Neuropathol. 2015 Oct;130(4):469-86
pubmed: 26296699
Autophagy. 2008 Jul;4(5):701-3
pubmed: 18414043
Int J Mol Sci. 2015 Mar 11;16(3):5517-27
pubmed: 25768338
Tissue Barriers. 2016 Jan 28;4(1):e1143544
pubmed: 27141424
Ann Neurol. 1987 Feb;21(2):159-70
pubmed: 3827224
J Neuropathol Exp Neurol. 2005 May;64(5):412-9
pubmed: 15892299
Hum Mol Genet. 2008 Jun 1;17(11):1540-55
pubmed: 18276609
Neuron. 2014 Feb 5;81(3):588-602
pubmed: 24507193
Neuroradiology. 1995 Nov;37(8):679-86
pubmed: 8748906
Am J Hum Genet. 2011 Apr 8;88(4):422-32
pubmed: 21419380
Semin Immunopathol. 2009 Nov;31(4):497-511
pubmed: 19779720
Lab Chip. 2018 Jan 16;18(2):249-270
pubmed: 29143053
AJNR Am J Neuroradiol. 2007 Jun-Jul;28(6):999
pubmed: 17569945
Am J Pathol. 2021 Nov;191(11):1856-1870
pubmed: 33895122
Nat Neurosci. 2012 Jul 26;15(8):1074-7
pubmed: 22837037
Nature. 2014 Apr 3;508(7494):55-60
pubmed: 24670647
Nat Rev Neurosci. 2006 Jan;7(1):41-53
pubmed: 16371949
Am J Med Genet A. 2015 Feb;167A(2):296-312
pubmed: 25604658
Nature. 2014 Nov 13;515(7526):264-8
pubmed: 25391964
Microvasc Res. 1999 Nov;58(3):312-28
pubmed: 10527772
Fluids Barriers CNS. 2019 Jun 6;16(1):15
pubmed: 31167667
PLoS One. 2011 Jan 10;6(1):e15846
pubmed: 21264342
Eur J Pharm Biopharm. 2014 Aug;87(3):409-32
pubmed: 24686194
PLoS One. 2014 Dec 23;9(12):e115981
pubmed: 25535736
Ann Clin Transl Neurol. 2015 Jul;2(7):774-9
pubmed: 26273690
Neurology. 2019 Mar 12;92(11):e1225-e1237
pubmed: 30737337
Biomed Mater. 2017 Mar 24;12(2):025014
pubmed: 28233757
Neuropediatrics. 1981 Nov;12(4):382-91
pubmed: 6801536
Psychiatr Genet. 2007 Apr;17(2):85-91
pubmed: 17413447
J Biol Chem. 2012 Nov 30;287(49):41374-85
pubmed: 23043099
Mol Aspects Med. 2012 Oct-Dec;33(5-6):579-89
pubmed: 22387049
AJNR Am J Neuroradiol. 2005 Mar;26(3):442-6
pubmed: 15760847
Pediatr Dev Pathol. 2009 May-Jun;12(3):180-6
pubmed: 18821826
Front Cell Neurosci. 2019 Sep 20;13:405
pubmed: 31616251
Brain Res Bull. 2016 Apr;122:1-11
pubmed: 26708939
Fluids Barriers CNS. 2018 Apr 23;15(1):12
pubmed: 29688865
Biomicrofluidics. 2019 Nov 22;13(6):064119
pubmed: 31768205
Neuron. 2016 Jan 6;89(1):37-53
pubmed: 26687838
Front Mol Biosci. 2016 Oct 25;3:68
pubmed: 27826550
Arterioscler Thromb Vasc Biol. 2008 Dec;28(12):2216-24
pubmed: 18818417
J Cereb Blood Flow Metab. 2016 Feb;36(2):451-5
pubmed: 26661200
Neurology. 2012 Aug 7;79(6):566-74
pubmed: 22843259
Brain Pathol. 1995 Jul;5(3):267-73
pubmed: 8520726
Curr Opin Cell Biol. 2015 Feb;32:121-30
pubmed: 25726916
J Neurosci Methods. 2001 May 30;107(1-2):15-23
pubmed: 11389937
J Neurol Sci. 1988 Apr;84(2-3):201-8
pubmed: 2837539
Neurology. 1976 Jul;26(7):607-14
pubmed: 180453
Nat Neurosci. 2021 Sep;24(9):1198-1209
pubmed: 34354283
Physiol Rev. 2013 Oct;93(4):1543-62
pubmed: 24137016
Nat Rev Neurosci. 2011 May;12(5):269-83
pubmed: 21505516
Cell. 2016 May 19;165(5):1238-1254
pubmed: 27118425
Neurochem Res. 2017 Sep;42(9):2519-2536
pubmed: 28634726
EMBO Rep. 2019 Nov 5;20(11):e48070
pubmed: 31617312
Free Radic Biol Med. 1996;21(6):871-88
pubmed: 8902532
Nat Rev Neurosci. 2013 May;14(5):311-21
pubmed: 23595014
Biochem J. 1984 Apr 1;219(1):1-14
pubmed: 6326753
Mol Neurodegener. 2019 Dec 2;14(1):45
pubmed: 31791377
Glia. 2008 Apr 15;56(6):633-45
pubmed: 18293412
Best Pract Res Clin Endocrinol Metab. 2015 Mar;29(2):261-73
pubmed: 25987178
Neuropediatrics. 2016 Dec;47(6):349-354
pubmed: 27564080
Brain Res. 1999 Jul 17;835(1):46-61
pubmed: 10448195
Hum Mol Genet. 2008 Dec 1;17(23):3728-39
pubmed: 18757878
Front Neurosci. 2019 Jun 21;13:582
pubmed: 31293366
Nat Biotechnol. 2014 Aug;32(8):760-72
pubmed: 25093883
J Biol Chem. 2010 Apr 2;285(14):10527-37
pubmed: 20110364
J Neurosci. 2012 Nov 14;32(46):16435-48
pubmed: 23152626
J Cell Sci. 2015 Jul 15;128(14):2415-22
pubmed: 26116570
Hum Mol Genet. 2003 Jul 1;12(13):1543-54
pubmed: 12812982
J Comp Neurol. 2001 Jul 23;436(2):167-81
pubmed: 11438922
Acta Neuropathol. 1983;61(1):36-42
pubmed: 6624384
J Cell Biol. 2017 Jan 2;216(1):31-40
pubmed: 28031422
Neuron. 2010 Jun 10;66(5):646-61
pubmed: 20547124
Neuroscience. 2015 Oct 1;305:86-98
pubmed: 26254240
PLoS One. 2017 Jan 4;12(1):e0169506
pubmed: 28052116
Lab Chip. 2018 Mar 13;18(6):851-860
pubmed: 29437173
Mech Dev. 2001 Oct;108(1-2):161-4
pubmed: 11578869
Stem Cell Rev Rep. 2021 Oct;17(5):1855-1873
pubmed: 33982246
J Immunol. 2014 Mar 15;192(6):2551-63
pubmed: 24510966
Front Cell Dev Biol. 2019 May 09;7:76
pubmed: 31143763
Pediatr Neurol. 1998 Apr;18(4):334-7
pubmed: 9588530
Radiology. 2006 Nov;241(2):510-7
pubmed: 17057071
Expert Opin Drug Metab Toxicol. 2016 May;12(5):499-508
pubmed: 26998936
Brain Pathol. 2018 May;28(3):399-407
pubmed: 29740948
Cell. 2011 Jul 8;146(1):18-36
pubmed: 21729779
Am J Hum Genet. 2007 Apr;80(4):811-5
pubmed: 17357087
Science. 1999 Apr 30;284(5415):770-6
pubmed: 10221902
Tissue Barriers. 2019;7(4):1651157
pubmed: 31505997
Nat Rev Drug Discov. 2016 Nov;15(11):751-769
pubmed: 27616293
Am J Hum Genet. 2001 Apr;68(4):831-8
pubmed: 11254442
Cell. 2011 Jun 10;145(6):831-4
pubmed: 21663789
Neuroscientist. 2011 Aug;17(4):423-36
pubmed: 21518814
PLoS Biol. 2020 May 13;18(5):e3000705
pubmed: 32401820
Proc Natl Acad Sci U S A. 2000 May 9;97(10):5621-6
pubmed: 10792049
Acta Neuropathol. 2004 Jun;107(6):481-8
pubmed: 15067553
Acta Neuropathol. 2017 Sep;134(3):351-382
pubmed: 28638987
Lancet Neurol. 2014 Oct;13(10):1045-60
pubmed: 25231526
Trends Cell Biol. 2004 Nov;14(11):628-38
pubmed: 15519852
J Neurophysiol. 2016 Nov 1;116(5):2093-2104
pubmed: 27512019
Neurology. 2000 Jan 25;54(2):463-8
pubmed: 10668715
Neurology. 2009 Feb 24;72(8):750-9
pubmed: 19237705
Curr Stem Cell Rep. 2016;2:236-247
pubmed: 27547709
Cell. 2007 Nov 30;131(5):861-72
pubmed: 18035408
AJNR Am J Neuroradiol. 2012 Oct;33(9):1731-9
pubmed: 22538069
Nat Protoc. 2010 Jul;5(7):1265-72
pubmed: 20595955
Cell Stem Cell. 2008 Nov 6;3(5):519-32
pubmed: 18983967
Brain Struct Funct. 2020 Mar;225(2):805-816
pubmed: 32072250
Pediatr Neurol. 2009 May;40(5):380-2
pubmed: 19380076
Clin Genet. 2016 Oct;90(4):293-304
pubmed: 27234264
Acta Neuropathol. 2018 Mar;135(3):311-336
pubmed: 29411111
J Cell Physiol. 2007 Jul;212(1):244-51
pubmed: 17323381
Nat Commun. 2018 Oct 9;9(1):4167
pubmed: 30301888
Cell Rep. 2015 Feb 3;10(4):537-50
pubmed: 25640179
Acta Neuropathol. 2006 Apr;111(4):300-11
pubmed: 16523341
Arch Med Res. 2014 Nov;45(8):687-97
pubmed: 25431839
Blood. 2010 Aug 5;116(5):829-40
pubmed: 20404134
Nat Rev Immunol. 2012 Sep;12(9):623-35
pubmed: 22903150
Biosens Bioelectron. 2015 Jan 15;63:218-231
pubmed: 25105943
Acta Neuropathol. 1996 Aug;92(2):206-12
pubmed: 8841668
AJNR Am J Neuroradiol. 2009 Jun;30(6):1244-7
pubmed: 19270103
Brain. 2013 Jan;136(Pt 1):245-58
pubmed: 23365100
Nat Commun. 2020 May 1;11(1):2138
pubmed: 32358570
Brain Pathol. 2014 Sep;24(5):525-44
pubmed: 25323668
Cell Rep. 2018 Jul 31;24(5):1203-1217.e6
pubmed: 30067976
J Am Heart Assoc. 2016 Nov 4;5(11):
pubmed: 27815267
Nature. 2010 Sep 16;467(7313):285-90
pubmed: 20644535
Ann Neurol. 2014 Jul;76(1):5-19
pubmed: 24916848
J Neuroimaging. 2014 Sep-Oct;24(5):444-52
pubmed: 23751174
Ann Clin Transl Neurol. 2018 May 21;5(7):788-802
pubmed: 30009197
J Biol Chem. 2006 Dec 15;281(50):38634-43
pubmed: 17038307
Lancet Neurol. 2013 Nov;12(11):1096-105
pubmed: 24074724
J Cell Sci. 2009 Nov 1;122(Pt 21):3942-53
pubmed: 19825935
Acta Neuropathol. 2004 Apr;107(4):311-8
pubmed: 14735305
Neuropediatrics. 1984 Sep;15 Suppl:62-5
pubmed: 6546165
Neuroradiology. 1991;33(6):478-93
pubmed: 1780048
Curr Opin Biomed Eng. 2018 Mar;5:6-12
pubmed: 29915815
Acta Neuropathol Commun. 2017 Mar 31;5(1):27
pubmed: 28359321
Glia. 2008 Apr;56(5):568-78
pubmed: 18240301
Front Cell Neurosci. 2021 Jun 30;15:696540
pubmed: 34276312
Int J Neurosci. 2001 Aug;108(1-2):109-26
pubmed: 11328706
Adv Cancer Res. 2015;125:43-70
pubmed: 25640266
Nat Genet. 2001 Jan;27(1):117-20
pubmed: 11138011
Cardiovasc Psychiatry Neurol. 2011;2011:646958
pubmed: 21350722
Cell. 2019 Oct 3;179(2):292-311
pubmed: 31585077