GPR56-Regulated Granule Cell Adhesion Is Essential for Rostral Cerebellar Development

Koirala, Samir; Jin, Zhaohui; Piao, Xiangshu; Corfas, Gabriel

doi:10.1523/jneurosci.1182-09.2009

Cited by 88 publications

(101 citation statements)

References 50 publications

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“…In dystroglycanopathies and GPR56 mutation, migration of neurons through the hindbrain basement membranes is also defective (Muntoni et al 2004;Koirala et al 2009). …”

Section: Overmigration Syndromesmentioning

confidence: 99%

“…The cobblestone complex appears to be due to defective glycosylation of alphadystroglycan, while migrating cerebellar granule cells of GPR56 mutant mice, which have malformations of the rostral cerebellum, show selective deficiency in adhesion to laminin-1 and fibronectin but normal adhesion to collagen IV (Koirala et al 2009). …”

Section: Disruption Of Hindbrain Migration Pathwaysmentioning

confidence: 99%

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Abnormal development of the human cerebral cortex

Squier

Jansen

2010

Journal of Anatomy

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This review presents an overview of human cortical malformation based on the insights gained from examination of human fetal brains. Examination at early stages of fetal brain development allows the identification of the specific pathways which are disrupted in human cortical malformation. Detailed examination of human fetal brains in parallel with studies of genetics and animal models is leading to new concepts of cortical malformations. Here we review a range of human cortical malformations based on a simple classification according to the developmental process thought to be disrupted: neuroblast proliferation, undermigration, overmigration, cortical maturation and destructive lesions. A single case example of a dated intrauterine injury illustrates the spectrum of malformations which may result at a single period in development. The recommended methods of examination of human fetal brain are described together with some of their pitfalls. Detailed neuropathological observations indicate the need for caution in the classification of malformations; radiological findings and pathology of the mature brain do not reflect the specific disruptive pathways of cortical malformations. While many insults may lead to the same pattern of malformation, a single insult can lead to multiple patterns of malformation. Our detailed studies of the human fetal brain suggest that the interface between the meninges and the radial glial end feet may be an intriguing new focus of interest in understanding cortical development.

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“…In dystroglycanopathies and GPR56 mutation, migration of neurons through the hindbrain basement membranes is also defective (Muntoni et al 2004;Koirala et al 2009). …”

Section: Overmigration Syndromesmentioning

confidence: 99%

Section: Disruption Of Hindbrain Migration Pathwaysmentioning

confidence: 99%

Abnormal development of the human cerebral cortex

Squier

Jansen

2010

Journal of Anatomy

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“…Subsequently identified aGPCR ligands were dermatan sulfate, a5b1 integrin, tissue transglutaminase 2, phosphatidylserine, LPS, C1q, lasso/teneurin-2, collagen III, and Thy-1/CD90 (12)(13)(14)(15)(16)(17)(18)(19)(20). Evidence was obtained that aGPCRs have a role in cell positioning and tissue organization in various organ systems (21,22); however, in the strictest sense, aGPCRs are still functional orphans. The main problem is a lack of understanding of how these atypical GPCRs are activated.…”

mentioning

confidence: 99%

Shear Stress–Dependent Downregulation of the Adhesion-G Protein–Coupled Receptor CD97 on Circulating Leukocytes upon Contact with Its Ligand CD55

Karpus

Veninga

Hoek

et al. 2013

The Journal of Immunology

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Adhesion G protein–coupled receptors (aGPCRs) are two-subunit molecules, consisting of an adhesive extracellular α subunit that couples noncovalently to a seven-transmembrane β subunit. The cooperation between the two subunits and the effect of endogenous ligands on the functioning of aGPCRs is poorly understood. In this study, we investigated the interaction between the pan-leukocyte aGPCR CD97 and its ligand CD55. We found that leukocytes from CD55-deficient mice express significantly increased levels of cell surface CD97 that normalized after transfer into wild-type mice because of contact with CD55 on both leukocytes and stromal cells. Downregulation of both CD97 subunits occurred within minutes after first contact with CD55 in vivo, which correlated with an increase in plasma levels of soluble CD97. In vitro, downregulation of CD97 on CD55-deficient leukocytes cocultured with wild-type blood cells was strictly dependent on shear stress. In vivo, CD55-mediated downregulation of CD97 required an intact circulation and was not observed on cells that lack contact with the blood stream, such as microglia. Notably, de novo ligation of CD97 did not activate signaling molecules constitutively engaged by CD97 in cancer cells, such as ERK and protein kinase B/Akt. We conclude that CD55 downregulates CD97 surface expression on circulating leukocytes by a process that requires physical forces, but based on current evidence does not induce receptor signaling. This regulation can restrict CD97–CD55-mediated cell adhesion to tissue sites.

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“…Recently, GPR56 was found to be expressed in a subset of cytotoxic NK cells (9). In CNS, GPR56 is essential for the normal development of cerebral cortex and proper cerebellar morphogenesis (10,11). Specifically, loss-of-function GPR56 mutations were identified as the sole cause of bilateral frontoparietal polymicrogyria (BFPP), a rare hereditary congenital disorder in human (12).…”

mentioning

confidence: 99%

Disease-associated GPR56 Mutations Cause Bilateral Frontoparietal Polymicrogyria via Multiple Mechanisms

Chiang

Hsiao

Huang

et al. 2011

Journal of Biological Chemistry

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Loss-of-function mutations in the gene encoding G proteincoupled receptor 56 (GPR56) lead to bilateral frontoparietal polymicrogyria (BFPP), an autosomal recessive disorder affecting brain development. The GPR56 receptor is a member of the adhesion-GPCR family characterized by the chimeric composition of a long ectodomain (ECD), a GPCR proteolysis site (GPS), and a sevenpass transmembrane (7TM) moiety. Interestingly, all identified BFPP-associated missense mutations are located within the extracellular region of GPR56 including the ECD, GPS, and the extracellular loops of 7TM. In the present study, a detailed molecular and functional analysis of the wild-type GPR56 and BFPP-associated point mutants shows that individual GPR56 mutants most likely cause BFPP via different combination of multiple mechanisms. These include reduced surface receptor expression, loss of GPS proteolysis, reduced receptor shedding, inability to interact with a novel protein ligand, and differential distribution of the 7TM moiety in lipid rafts. These results provide novel insights into the cellular functions of GPR56 receptor and reveal molecular mechanisms whereby GPR56 mutations induce BFPP.

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GPR56-Regulated Granule Cell Adhesion Is Essential for Rostral Cerebellar Development

Cited by 88 publications

References 50 publications

Abnormal development of the human cerebral cortex

Abnormal development of the human cerebral cortex

Shear Stress–Dependent Downregulation of the Adhesion-G Protein–Coupled Receptor CD97 on Circulating Leukocytes upon Contact with Its Ligand CD55

Disease-associated GPR56 Mutations Cause Bilateral Frontoparietal Polymicrogyria via Multiple Mechanisms

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