Astrocytes in the regulation of cerebrovascular functions

M Cohen‐Salmon, L Slaoui, N Mazaré, A Gilbert… - Glia, 2021 - Wiley Online Library
Astrocytes are the most numerous type of neuroglia in the brain and have a predominant
influence on the cerebrovascular system; they control perivascular homeostasis, the integrity …

Chloride channels in astrocytes: structure, roles in brain homeostasis and implications in disease

X Elorza-Vidal, H Gaitán-Peñas, R Estévez - International journal of …, 2019 - mdpi.com
Astrocytes are the most abundant cell type in the CNS (central nervous system). They exert
multiple functions during development and in the adult CNS that are essential for brain …

Mlc1-Expressing Perivascular Astrocytes Promote Blood–Brain Barrier Integrity

JE Morales, A De, AA Miller, Z Chen… - Journal of …, 2022 - Soc Neuroscience
In the mammalian brain, perivascular astrocytes (PAs) closely juxtapose blood vessels and
are postulated to have important roles in the control of vascular physiology, including …

Megalencephalic leukoencephalopathy with subcortical cysts is a developmental disorder of the gliovascular unit

A Gilbert, X Elorza-Vidal, A Rancillac, A Chagnot… - Elife, 2021 - elifesciences.org
Absence of the astrocyte-specific membrane protein MLC1 is responsible for
megalencephalic leukoencephalopathy with subcortical cysts (MLC), a rare type of …

Antisense oligonucleotide reverses leukodystrophy in Canavan disease mice

V Hull, Y Wang, T Burns, S Zhang… - Annals of …, 2020 - Wiley Online Library
Marked elevation in the brain concentration of N‐acetyl‐L‐aspartate (NAA) is a
characteristic feature of Canavan disease, a vacuolar leukodystrophy resulting from …

GlialCAM/MLC1 modulates LRRC8/VRAC currents in an indirect manner: Implications for megalencephalic leukoencephalopathy

X Elorza-Vidal, S Sirisi, H Gaitan-Penas… - Neurobiology of …, 2018 - Elsevier
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare type of
leukodystrophy caused by mutations in either MLC1 or GLIALCAM genes. Previous work …

Astrocytic modulation of potassium under seizures

F Wang, X Qi, J Zhang, JH Huang - Neural regeneration research, 2020 - journals.lww.com
The contribution of an impaired astrocytic K+ regulation system to epileptic neuronal
hyperexcitability has been increasingly recognized in the last decade. A defective K+ …

Megalencephalic leukoencephalopathy with subcortical cysts protein-1 (MLC1) counteracts astrocyte activation in response to inflammatory signals

MS Brignone, A Lanciotti, B Serafini, C Mallozzi… - Molecular …, 2019 - Springer
Megalencephalic leukoencephalopathy with subcortical cysts protein-1 (MLC1) is a
membrane protein expressed by perivascular astrocytes. MLC1 mutations cause MLC, an …

Megalencephalic leukoencephalopathy: insights into pathophysiology and perspectives for therapy

A Bosch, R Estévez - Frontiers in cellular neuroscience, 2021 - frontiersin.org
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare genetic
disorder belonging to the group of vacuolating leukodystrophies. It is characterized by …

Structural basis for the dominant or recessive character of GLIALCAM mutations found in leukodystrophies

X Elorza-Vidal, E Xicoy-Espaulella… - Human Molecular …, 2020 - academic.oup.com
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a type of
leukodystrophy characterized by white matter edema, and it is caused mainly by recessive …