Subcommissural organ, cerebrospinal fluid circulation, and hydrocephalus

Pérez-Fígares, J. M.; Jiménez, Antonio J.; Rodríguez, Estéban M.

doi:10.1002/1097-0029(20010301)52:5<591::aid-jemt1043>3.0.co;2-7

Cited by 93 publications

(87 citation statements)

References 118 publications

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“…Abnormalities of the SCO have RFX4 and brain development been associated with hydrocephalus in many studies, as recently reviewed (Perez-Figares et al, 2001). Although there has been some debate in the literature about whether the SCO abnormalities cause or are consequences of the hydrocephalus, the overall consensus seems to be in favor of the SCO abnormalities preceding and causing the hydrocephalus, owing to effective stenosis of the aqueduct of Sylvius.…”

Section: Discussionmentioning

confidence: 99%

“…3A). This organ produces Reissner's fibers, and both the organ and the fibers have been shown to be important for the patency of the aqueduct, in that destruction of the SCO leads to obstructive hydrocephalus (Perez-Figares et al, 2001). Antibodies specific to Reissner's fibers (Rodriguez et al, 1984;Rodriguez et al, 2001; strongly and specifically labeled the SCO from the wild-type mice (Fig.…”

Section: Evaluation Of Transgenic Micementioning

confidence: 92%

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Graded phenotypic response to partial and complete deficiency of a brain-specific transcript variant of the winged helix transcription factor RFX4

et al. 2003

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Interruption of a single allele prevented formation of the subcommissural organ, a structure important for cerebrospinal fluid flow through the aqueduct of Sylvius, and resulted in congenital hydrocephalus. These data implicate the RFX4_v3 variant transcript as being crucial for early brain development, as well as for the genesis of the subcommissural organ. These findings may be relevant to human congenital hydrocephalus, a birth defect that affects ~0.6 per 1000 newborns.

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Section: Discussionmentioning

confidence: 99%

Section: Evaluation Of Transgenic Micementioning

confidence: 92%

Graded phenotypic response to partial and complete deficiency of a brain-specific transcript variant of the winged helix transcription factor RFX4

et al. 2003

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Section: Introductionmentioning

confidence: 99%

“…The defect may be accompanied by an obstructed aqueduct of Sylvius, the narrow channel connecting the third and fourth brain ventricles (noncommunicating hydrocephalus), or by a normal aqueduct (communicating hydrocephalus). The pathogenesis of most cases of communicating hydrocephalus is largely unknown (for reviews, see Perez-Figares et al, 2001;Crews et al, 2004).Nonmuscle myosin (NM) II, one of the major cytoskeletal motor proteins, plays an important role in cell migration (Svitkina et al, 1997;Ma et al, 2004;Even-Ram et al, 2007;Vicente-Manzanares et al, 2007), cell-cell adhesion Shewan et al, 2005;Giannone et al, 2007), and cell division (De Lozanne and Spudich, 1987;Takeda et al, 2003;Bao et al, 2005). The molecular structure of NM II is a hexamer consisting of a pair of myosin heavy chains (200 kDa) and two pairs of light chains (20 and 17 kDa).…”

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confidence: 99%

Loss of Cell Adhesion Causes Hydrocephalus in Nonmuscle Myosin II-B–ablated and Mutated Mice

Bao

Adelstein

2007

MBoC

103

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Ablation of nonmuscle myosin (NM) II-B in mice during embryonic development leads to marked enlargement of the cerebral ventricles and destruction of brain tissue, due to hydrocephalus. We have identified a transient mesh-like structure present at the apical border of cells lining the spinal canal of mice during development. This structure, which only contains the II-B isoform of NM, also contains ␤-catenin and N-cadherin, consistent with a role in cell adhesion. Ablation of NM II-B or replacement of NM II-B with decreased amounts of a mutant (R709C), motor-impaired NM II-B in mice results in collapse of the mesh-like structure and loss of cell adhesion. This permits the underlying neuroepithelial cells to invade the spinal canal and obstruct cerebral spinal fluid flow. These defects in the CNS of NM II-B-ablated mice seem to be the cause of hydrocephalus. Interestingly, the mesh-like structure and patency of the spinal canal can be restored by increasing expression of the motor-impaired NM II-B, which also rescues hydrocephalus. However, the mutant isoform cannot completely rescue neuronal cell migration. These studies show that the scaffolding properties of NM II-B play an important role in cell adhesion, thereby preventing hydrocephalus during mouse brain development. INTRODUCTIONCongenital hydrocephalus affects one to three humans per 1000 live births. The results of this abnormality can be devastating in that severe, untreated hydrocephalus can destroy brain tissue and thereby lead to mental retardation. Hydrocephalus occurs when there is an increase in pressure in the ventricular chambers due to a blockage in the circulation of cerebral spinal fluid (CSF) or when there is an increase in production or decrease in the absorption of the CSF. The defect may be accompanied by an obstructed aqueduct of Sylvius, the narrow channel connecting the third and fourth brain ventricles (noncommunicating hydrocephalus), or by a normal aqueduct (communicating hydrocephalus). The pathogenesis of most cases of communicating hydrocephalus is largely unknown (for reviews, see Perez-Figares et al., 2001;Crews et al., 2004).Nonmuscle myosin (NM) II, one of the major cytoskeletal motor proteins, plays an important role in cell migration (Svitkina et al., 1997;Ma et al., 2004;Even-Ram et al., 2007;Vicente-Manzanares et al., 2007), cell-cell adhesion Shewan et al., 2005;Giannone et al., 2007), and cell division (De Lozanne and Spudich, 1987;Takeda et al., 2003;Bao et al., 2005). The molecular structure of NM II is a hexamer consisting of a pair of myosin heavy chains (200 kDa) and two pairs of light chains (20 and 17 kDa). In mammals, three isoforms of the nonmuscle myosin heavy chain (NMHC) have been identified, NMHC II-A, II-B, and II-C, encoded by three different genes, Myh 9, Myh 10, and Myh 14 in humans. The NMHCs share a 60 -80% identity in amino acids, but they show significant differences in their motor activities (Golomb et al., 2004;Kim et al., 2005). In general, all three isoforms are ubiquitously expressed in vertebrat...

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“…Obstruction usually occurs in the narrowed segments of the ventricular system, typically the cerebral aqueduct. Indeed, stenosis of the cerebral aqueduct is considered the primary cause of congenital hydrocephalus (1,2). Several factors play a role in the maintenance of CSF flow through the narrow canals, including ciliary movement on ependymal cells and a functioning subcommissural organ (SCO), an ependymal gland located in the dorsocaudal region of the third ventricle at the entrance of the Sylvian aqueduct (Figure 2) (3,4).…”

mentioning

confidence: 99%

Neuropeptide signaling and hydrocephalus: SCO with the flow

Picketts¹

2006

Journal of Clinical Investigation

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Subcommissural organ, cerebrospinal fluid circulation, and hydrocephalus

Cited by 93 publications

References 118 publications

Graded phenotypic response to partial and complete deficiency of a brain-specific transcript variant of the winged helix transcription factor RFX4

Graded phenotypic response to partial and complete deficiency of a brain-specific transcript variant of the winged helix transcription factor RFX4

Loss of Cell Adhesion Causes Hydrocephalus in Nonmuscle Myosin II-B–ablated and Mutated Mice

Neuropeptide signaling and hydrocephalus: SCO with the flow

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