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Article: Spatially and temporally distinct patterns of expression for VPS10P domain receptors in human cerebral organoids.

Febbraro, Fabia / Andersen, Helena Hørdum Breum / Kitt, Meagan M / Willnow, Thomas E

Frontiers in cell and developmental biology

2023 Volume 11, Page(s) 1229584

Abstract: Vacuolar protein sorting 10 protein (VPS10P) domain receptors are a unique class of intracellular sorting receptors that emerge as major risk factors associated with psychiatric and neurodegenerative diseases, including bipolar disorders, autism, ... ...

Abstract	Vacuolar protein sorting 10 protein (VPS10P) domain receptors are a unique class of intracellular sorting receptors that emerge as major risk factors associated with psychiatric and neurodegenerative diseases, including bipolar disorders, autism, schizophrenia, as well as Alzheimer's disease and frontotemporal dementia. Yet, the lack of suitable experimental models to study receptor functions in the human brain has hampered elucidation of receptor actions in brain disease. Here, we have adapted protocols using human cerebral organoids to the detailed characterization of VPS10P domain receptor expression during neural development and differentiation, including single-cell RNA sequencing. Our studies uncovered spatial and temporal patterns of expression unique to individual receptor species in the human brain. While
Language	English
Publishing date	2023-09-29
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2737824-X
ISSN	2296-634X
ISSN	2296-634X
DOI	10.3389/fcell.2023.1229584
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Article ; Online: Nanotubes, the fast track to treatment of Dent disease?

Willnow, Thomas E

Kidney international

2017 Volume 91, Issue 4, Page(s) 776–778

Abstract: Studies in mice have suggested bone marrow transplantation as a strategy to correct the endocytic dysfunction of the proximal tubules in renal Fanconi syndrome, yet the mode of action has remained mysterious. Using a mouse model of Dent disease, Gabriel ... ...

Abstract	Studies in mice have suggested bone marrow transplantation as a strategy to correct the endocytic dysfunction of the proximal tubules in renal Fanconi syndrome, yet the mode of action has remained mysterious. Using a mouse model of Dent disease, Gabriel et al. now show that rescue of the resorptive capacity in the diseased kidney involves cell-to-cell contact between engrafted and host cells via nanotubes, cellular projections that enable transfer of wild-type activity into mutant cells of the proximal tubule.
Language	English
Publishing date	2017-04
Publishing country	United States
Document type	Journal Article
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2016.12.030
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Zs.A 797: Show issues

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Article ; Online: VPS10P Domain Receptors: Sorting Out Brain Health and Disease.

Malik, Anna R / Willnow, Thomas E

Trends in neurosciences

2020 Volume 43, Issue 11, Page(s) 870–885

Abstract: VPS10P (vacuolar protein sorting 10 protein) domain receptors are neuronal sorting receptors that direct cargo proteins to their destined location in subcellular compartments of the soma, dendrites, and the axon. Protein sorting by receptors such as ... ...

Abstract	VPS10P (vacuolar protein sorting 10 protein) domain receptors are neuronal sorting receptors that direct cargo proteins to their destined location in subcellular compartments of the soma, dendrites, and the axon. Protein sorting by receptors such as SORLA, sortilin, and SorCS2 controls functional integrity and viability of neurons, whereas sorting receptor dysfunctions are linked to acute, psychiatric, and neurodegenerative diseases. Here, we discuss molecular mechanisms that define ligand repertoire and sorting path and that control plasticity of VPS10P domain receptor expression in the healthy brain and in response to injury. These findings highlight important concepts in neuronal protein sorting and why aberrant sorting contributes to the progression of devastating diseases of the human brain, including epilepsy, Alzheimer's disease, and frontotemporal dementia.
MeSH term(s)	Alzheimer Disease ; Brain ; Humans ; Ligands ; Neurons ; Protein Transport
Chemical Substances	Ligands
Language	English
Publishing date	2020-09-04
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	282488-7
ISSN	1878-108X ; 0378-5912 ; 0166-2236
ISSN (online)	1878-108X
ISSN	0378-5912 ; 0166-2236
DOI	10.1016/j.tins.2020.08.003
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Zs.A 1442: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MG 53: Show issues

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Article ; Online: Excitatory Amino Acid Transporters in Physiology and Disorders of the Central Nervous System.

Malik, Anna R / Willnow, Thomas E

International journal of molecular sciences

2019 Volume 20, Issue 22

Abstract: Excitatory amino acid transporters (EAATs) encompass a class of five transporters with distinct expression in neurons and glia of the central nervous system (CNS). EAATs are mainly recognized for their role in uptake of the amino acid glutamate, the ... ...

Abstract	Excitatory amino acid transporters (EAATs) encompass a class of five transporters with distinct expression in neurons and glia of the central nervous system (CNS). EAATs are mainly recognized for their role in uptake of the amino acid glutamate, the major excitatory neurotransmitter. EAATs-mediated clearance of glutamate released by neurons is vital to maintain proper glutamatergic signalling and to prevent toxic accumulation of this amino acid in the extracellular space. In addition, some EAATs also act as chloride channels or mediate the uptake of cysteine, required to produce the reactive oxygen speciesscavenger glutathione. Given their central role in glutamate homeostasis in the brain, as well as their additional activities, it comes as no surprise that EAAT dysfunctions have been implicated in numerous acute or chronic diseases of the CNS, including ischemic stroke and epilepsy, cerebellar ataxias, amyotrophic lateral sclerosis, Alzheimer's disease and Huntington's disease. Here we review the studies in cellular and animal models, as well as in humans that highlight the roles of EAATs in the pathogenesis of these devastating disorders. We also discuss the mechanisms regulating EAATs expression and intracellular trafficking and new exciting possibilities to modulate EAATs and to provide neuroprotection in course of pathologies affecting the CNS.
MeSH term(s)	Animals ; Biological Transport ; Brain Diseases/metabolism ; Brain Diseases/pathology ; Brain Diseases/physiopathology ; Central Nervous System/metabolism ; Central Nervous System/pathology ; Central Nervous System/physiopathology ; Glutamate Plasma Membrane Transport Proteins/metabolism ; Humans ; Neurons/metabolism ; Neurons/pathology ; Synaptic Transmission
Chemical Substances	Glutamate Plasma Membrane Transport Proteins
Language	English
Publishing date	2019-11-12
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms20225671
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Article ; Online: SORCS2 activity in pancreatic α-cells safeguards insulin granule formation and release from glucose-stressed β-cells.

Kalnytska, Oleksandra / Qvist, Per / Kunz, Séverine / Conrad, Thomas / Willnow, Thomas E / Schmidt, Vanessa

iScience

2023 Volume 27, Issue 1, Page(s) 108725

Abstract: Sorting receptor SORCS2 is a stress-response factor protecting neurons from acute insults, such as during epilepsy. SORCS2 is also expressed in the pancreas, yet its action in this tissue remains unknown. Combining metabolic studies in SORCS2-deficient ... ...

Abstract	Sorting receptor SORCS2 is a stress-response factor protecting neurons from acute insults, such as during epilepsy. SORCS2 is also expressed in the pancreas, yet its action in this tissue remains unknown. Combining metabolic studies in SORCS2-deficient mice with
Language	English
Publishing date	2023-12-13
Publishing country	United States
Document type	Journal Article
ISSN	2589-0042
ISSN (online)	2589-0042
DOI	10.1016/j.isci.2023.108725
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Article ; Online: LRP2 controls sonic hedgehog-dependent differentiation of cardiac progenitor cells during outflow tract formation.

Christ, Annabel / Marczenke, Maike / Willnow, Thomas E

Human molecular genetics

2020 Volume 29, Issue 19, Page(s) 3183–3196

Abstract: Conotruncal malformations are a major cause of congenital heart defects in newborn infants. Recently, genetic screens in humans and in mouse models have identified mutations in LRP2, a multi-ligand receptor, as a novel cause of a common arterial trunk, a ...

Abstract	Conotruncal malformations are a major cause of congenital heart defects in newborn infants. Recently, genetic screens in humans and in mouse models have identified mutations in LRP2, a multi-ligand receptor, as a novel cause of a common arterial trunk, a severe form of outflow tract (OFT) defect. Yet, the underlying mechanism why the morphogen receptor LRP2 is essential for OFT development remained unexplained. Studying LRP2-deficient mouse models, we now show that LRP2 is expressed in the cardiac progenitor niche of the anterior second heart field (SHF) that contributes to the elongation of the OFT during separation into aorta and pulmonary trunk. Loss of LRP2 in mutant mice results in the depletion of a pool of sonic hedgehog-dependent progenitor cells in the anterior SHF due to premature differentiation into cardiomyocytes as they migrate into the OFT myocardium. Depletion of this cardiac progenitor cell pool results in aberrant shortening of the OFT, the likely cause of CAT formation in affected mice. Our findings identified the molecular mechanism whereby LRP2 controls the maintenance of progenitor cell fate in the anterior SHF essential for OFT separation, and why receptor dysfunction is a novel cause of conotruncal malformation.
MeSH term(s)	Animals ; Cell Differentiation ; Cell Lineage ; Cell Movement ; Cell Proliferation ; Female ; Heart Defects, Congenital/etiology ; Heart Defects, Congenital/metabolism ; Heart Defects, Congenital/pathology ; Hedgehog Proteins/genetics ; Hedgehog Proteins/metabolism ; Low Density Lipoprotein Receptor-Related Protein-2/physiology ; Mice ; Mice, Knockout ; Morphogenesis ; Myocytes, Cardiac/metabolism ; Myocytes, Cardiac/pathology ; Signal Transduction ; Stem Cells/metabolism ; Stem Cells/pathology
Chemical Substances	Hedgehog Proteins ; Low Density Lipoprotein Receptor-Related Protein-2 ; Lrp2 protein, mouse ; Shh protein, mouse
Language	English
Publishing date	2020-09-03
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1108742-0
ISSN	1460-2083 ; 0964-6906
ISSN (online)	1460-2083
ISSN	0964-6906
DOI	10.1093/hmg/ddaa200
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Zs.A 3469: Show issues

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Article ; Online: LRP2 contributes to planar cell polarity-dependent coordination of motile cilia function.

Bunatyan, Lena / Margineanu, Anca / Boutin, Camille / Montcouquiol, Mireille / Bachmann, Sebastian / Ilsø Christensen, Erik / Willnow, Thomas E / Christ, Annabel

Cell and tissue research

2023 Volume 392, Issue 2, Page(s) 535–551

Abstract: Motile cilia are protruding organelles on specialized epithelia that beat in a synchronous fashion to propel extracellular fluids. Coordination and orientation of cilia beating on individual cells and across tissues is a complex process dependent on ... ...

Abstract	Motile cilia are protruding organelles on specialized epithelia that beat in a synchronous fashion to propel extracellular fluids. Coordination and orientation of cilia beating on individual cells and across tissues is a complex process dependent on planar cell polarity (PCP) signaling. Asymmetric sorting of PCP pathway components, essential to establish planar polarity, involves trafficking along the endocytic path, but the underlying regulatory processes remain incompletely understood. Here, we identified the endocytic receptor LRP2 as regulator of PCP component trafficking in ependyma, a multi-ciliated cell type that is involved in facilitating flow of the cerebrospinal fluid in the brain ventricular system. Lack of receptor expression in gene-targeted mice results in a failure to sort PCP core proteins to the anterior or posterior cell side and, consequently, in the inability to coordinate cilia arrangement and to aligned beating (loss of rotational and translational polarity). LRP2 deficiency coincides with a failure to sort NHERF1, a cytoplasmic LRP2 adaptor to the anterior cell side. As NHERF1 is essential to translocate PCP core protein Vangl2 to the plasma membrane, these data suggest a molecular mechanism whereby LRP2 interacts with PCP components through NHERF1 to control their asymmetric sorting along the endocytic path. Taken together, our findings identified the endocytic receptor LRP2 as a novel regulator of endosomal trafficking of PCP proteins, ensuring their asymmetric partition and establishment of translational and rotational planar cell polarity in the ependyma.
MeSH term(s)	Animals ; Mice ; Cilia/metabolism ; Cell Polarity ; Ependyma/metabolism ; Cerebral Ventricles/metabolism ; Carrier Proteins/metabolism ; Wnt Signaling Pathway ; Low Density Lipoprotein Receptor-Related Protein-2/metabolism
Chemical Substances	Carrier Proteins ; Lrp2 protein, mouse ; Low Density Lipoprotein Receptor-Related Protein-2
Language	English
Publishing date	2023-02-11
Publishing country	Germany
Document type	Journal Article
ZDB-ID	125067-x
ISSN	1432-0878 ; 0302-766X
ISSN (online)	1432-0878
ISSN	0302-766X
DOI	10.1007/s00441-023-03757-7
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Article ; Online: Endocytic receptor LRP2/megalin-of holoprosencephaly and renal Fanconi syndrome.

Willnow, Thomas E / Christ, Annabel

Pflugers Archiv : European journal of physiology

2017 Volume 469, Issue 7-8, Page(s) 907–916

Abstract: Megalin (or LRP2) is an endocytic receptor that plays a central role in embryonic development and adult tissue homeostasis. Loss of this receptor in congenital or acquired diseases results in multiple organ dysfunctions, including forebrain malformation ( ...

Abstract	Megalin (or LRP2) is an endocytic receptor that plays a central role in embryonic development and adult tissue homeostasis. Loss of this receptor in congenital or acquired diseases results in multiple organ dysfunctions, including forebrain malformation (holoprosencephaly) and renal reabsorption defects (renal Fanconi syndrome). Here, we describe current concepts of the mode of receptor action that include co-receptors and a repertoire of different ligands, and we discuss how these interactions govern functional integrity of the kidney and the brain, and cause disease when defective.
Language	English
Publishing date	2017-08
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	6380-0
ISSN	1432-2013 ; 0031-6768
ISSN (online)	1432-2013
ISSN	0031-6768
DOI	10.1007/s00424-017-1992-0
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Article ; Online: LRP2 controls sonic hedgehog-dependent differentiation of cardiac progenitor cells during outflow tract formation

Christ, Annabel / Willnow, Thomas E.

bioRxiv

Abstract: Conotruncal malformations are a major cause of congenital heart defects in newborn infants. Recently, genetic screens in humans and mouse models have identified mutations in LRP2 as a novel cause of a common arterial trunk, a severe form of outflow tract ...

Abstract	Conotruncal malformations are a major cause of congenital heart defects in newborn infants. Recently, genetic screens in humans and mouse models have identified mutations in LRP2 as a novel cause of a common arterial trunk, a severe form of outflow tract (OFT) defect. Yet, the underlying mechanism why the morphogen receptor LRP2 is essential for OFT development remained unexplained. Studying LRP2-deficient mouse models, we now show that LRP2 is expressed in the cardiac progenitor niche of the anterior second heart field (SHF) that contributes to elongation of the OFT during separation into aorta and pulmonary trunk. Loss of LRP2 in mutant mice results in depletion of a pool of sonic hedgehog-dependent progenitor cells in the SHF due to premature differentiation into cardiomyocytes as they migrate into the OFT myocardium. Depletion of this cardiac progenitor cell pool results in aberrant shortening of the OFT, the cause of CAT formation in affected mice. Our findings identified the molecular mechanism whereby LRP2 controls maintenance of progenitor cell fate in the anterior SHF essential for OFT separation, and why receptor dysfunction is a novel cause of conotruncal malformation.
Keywords	covid19
Publisher	BioRxiv
Document type	Article ; Online
DOI	10.1101/801910
Database	COVID19

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Article ; Online: Protein sorting gone wrong--VPS10P domain receptors in cardiovascular and metabolic diseases.

Schmidt, Vanessa / Willnow, Thomas E

Atherosclerosis

2016 Volume 245, Page(s) 194–199

Abstract: VPS10P domain receptors are a unique class of sorting receptors that direct intracellular transport of target proteins in neurons and that play central roles in neurodegenerative processes. Surprisingly, genome-wide association studies now implicate the ... ...

Abstract	VPS10P domain receptors are a unique class of sorting receptors that direct intracellular transport of target proteins in neurons and that play central roles in neurodegenerative processes. Surprisingly, genome-wide association studies now implicate the very same receptors in cardiovascular and metabolic disturbances. In this review, we discuss current findings that uncovered some of the molecular mechanisms whereby sorting receptors, such as SORLA, sortilin, and SORCS1 control homeostasis in cardiovascular and metabolic tissues, and how they promote hypercholesterolemia, atherosclerosis, obesity, and diabetes, when being altered.
MeSH term(s)	Cardiovascular Diseases/genetics ; Cardiovascular Diseases/metabolism ; Genome-Wide Association Study ; Humans ; Metabolic Diseases/genetics ; Metabolic Diseases/metabolism ; Protein Transport ; Receptors, Cell Surface/genetics
Chemical Substances	Receptors, Cell Surface
Language	English
Publishing date	2016-02
Publishing country	Ireland
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	80061-2
ISSN	1879-1484 ; 0021-9150
ISSN (online)	1879-1484
ISSN	0021-9150
DOI	10.1016/j.atherosclerosis.2015.11.027
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Zs.A 226: Show issues

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