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Roundabout, axon guidance receptor, homolog 3

Robo3, Rig-1
This gene is a member of the Roundabout (ROBO) gene family that controls neurite outgrowth, growth cone guidance, and axon fasciculation. ROBO proteins are a subfamily of the immunoglobulin transmembrane receptor superfamily. SLIT proteins 1-3, a family of secreted chemorepellants, are ligands for ROBO proteins and SLIT/ROBO interactions regulate myogenesis, leukocyte migration, kidney morphogenesis, angiogenesis, and vasculogenesis in addition to neurogenesis. This gene, ROBO3, has a putative extracellular domain with five immunoglobulin (Ig)-like loops and three fibronectin (Fn) type III motifs, a transmembrane segment, and a cytoplasmic tail with three conserved signaling motifs: CC0, CC2, and CC3 (CC for conserved cytoplasmic). Unlike other ROBO family members, ROBO3 lacks motif CC1. The ROBO3 gene regulates axonal navigation at the ventral midline of the neural tube. In mouse, loss of Robo3 results in a complete failure of commissural axons to cross the midline throughout the spinal cord and the hindbrain. Mutations ROBO3 result in horizontal gaze palsy with progressive scoliosis (HGPPS); an autosomal recessive disorder characterized by congenital absence of horizontal gaze, progressive scoliosis, and failure of the corticospinal and somatosensory axon tracts to cross the midline in the medulla. Alternative transcript variants have been described but have not been experimentally validated. [provided by RefSeq, Dec 2009] (from NCBI)
Top mentioned proteins: Robo1, Robo2, Midline, Slit2, HAD
Papers on Robo3
ROBO3 promotes growth and metastasis of pancreatic carcinoma.
New
Li et al., Shanghai, China. In Cancer Lett, Oct 2015
Here we showed that ROBO3 expression is up-regulated in pancreatic cancer tissue samples and cell lines.
New insights into implication of the SLIT/ROBO pathway in the prehierarchical follicle development of hen ovary.
New
Xu et al., Changchun, China. In Poult Sci, Sep 2015
The aims of the present study were to examine the spatial and temporal expression of the SLIT ligand genes (SLIT1, SLIT2, and SLIT3) and their receptor ROBO1, ROBO2, ROBO3, and ROBO4 genes in various-sized prehierarchical follicles during hen ovary development and the effects of activin A and inhibin A on the expression of these genes in the cultured hen follicles.
Ipsilateral hemiparesis caused by putaminal hemorrhage in a patient with horizontal gaze palsy with progressive scoliosis: a case report.
Kanemura et al., Ōsaka, Japan. In Bmc Neurol, 2014
BACKGROUND: Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disorder caused by mutations in the ROBO3 gene, resulting in a critical absence of crossing fibers in the brainstem.
Longitudinal assessment of DNA methylation changes during HPVE6E7-induced immortalization of primary keratinocytes.
Steenbergen et al., Amsterdam, Netherlands. In Epigenetics, 2014
In this study, cells were monitored for hypermethylation of 14 host genes (APC, CADM1, CYGB, FAM19A4, hTERT, mir124-1, mir124-2, mir124-3, MAL, PHACTR3, PRDM14, RASSF1A, ROBO3, and SFRP2) at 4 different stages during immortalization.
The ECEL1-related strabismus phenotype is consistent with congenital cranial dysinnervation disorder.
Alkuraya et al., Riyadh, Saudi Arabia. In J Aapos, 2014
CCDDs have been associated with dominant or recessive monogenic mutations in at least 7 different genes (CHN1, SALL4, HOXA1, KIF21A, PHOX2A, TUBB3, ROBO3) that cause phenotypes such as Duane retraction syndrome, congenital fibrosis of the extraocular muscles, and horizontal gaze palsy with progressive scoliosis.
Infantile esotropia with cross-fixation, inability to abduct, and underlying horizontal gaze palsy with progressive scoliosis.
Abu-Amero et al., Riyadh, Saudi Arabia. In J Aapos, 2014
We report the case of a 10-month-old girl with cross-fixation and inability to abduct who was genetically proven to have horizontal gaze palsy with progressive scoliosis (recessive ROBO3 mutations).
Radiological features of horizontal gaze palsy with progressive scoliosis. An 'Aunt Minnie' diagnosis?
Maller et al., In Del Med J, 2014
Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare autosomal recessive disorder characterized by congenital absence of normal horizontal eye movements and progressive scoliosis through childhood and adolescence.
Early aberrant DNA methylation events in a mouse model of acute myeloid leukemia.
Plass et al., Heidelberg, Germany. In Genome Med, 2013
Nine novel hypermethylated genes, FZD5, FZD8, PRDM16, ROBO3, CXCL14, BCOR, ITPKA, HES6 and TAL1, the latter four being potential PU.1 targets, were confirmed to be hypermethylated in human normal karyotype AML patients, underscoring the relevance of the mouse model for human AML.
Horizontal Gaze Palsy With Progressive Scoliosis and Severe Keratoconus With a Compound Heterozygous Mutation in ROBO3.
Santos et al., In J Pediatr Ophthalmol Strabismus, 2013
ROBO3 analysis identified compound heterozygous mutations.
Slit2 and Robo3 modulate the migration of GnRH-secreting neurons.
GeneRIF
Parnavelas et al., London, United Kingdom. In Development, 2012
Data show that the gooseberry/gooseberry-neuro (gsb/gsb-n) transcription factor genes act to specify MP3 cell fate.
Slit/Robo-mediated axon guidance in Tribolium and Drosophila: divergent genetic programs build insect nervous systems.
GeneRIF
Bashaw et al., Philadelphia, United States. In Dev Biol, 2012
Data show that Robo and Robo2 promote midline repulsion, while Robo2 and Robo3 specify the position of longitudinal axon pathways.
Slit1b-Robo3 signaling and N-cadherin regulate apical process retraction in developing retinal ganglion cells.
GeneRIF
Harris et al., Cambridge, United Kingdom. In J Neurosci, 2012
Slit-Robo signaling downregulates N-cadherin activity to allow apical retraction in newly generated retinal ganglion cells.
Horizontal gaze palsy and progressive scoliosis due to a deleterious mutation in ROBO3.
GeneRIF
Bosley et al., Riyadh, Saudi Arabia. In Ophthalmic Genet, 2011
The novel ROBO3 mutation in this family may be among the most deleterious
Horizontal gaze palsy with progressive scoliosis: three novel ROBO3 mutations and descriptions of the phenotypes of four patients.
GeneRIF
Neugebauer et al., Köln, Germany. In Mol Vis, 2010
Three novel ROBO3 mutations have been identified in consanguineous patients with horizontal gaze palsy and progressive scoliosis.
The genetic basis of complex strabismus.
Review
Engle, Boston, United States. In Pediatr Res, 2006
We are discovering that these disorders result from mutations in genes necessary for the normal development and connectivity of brainstem ocular motoneurons, including PHOX2A, SALL4, KIF21A, ROBO3, and HOXA1, and we now refer to these syndromes as the "congenital cranial dysinnervation disorders," or CCDD.
Mutations in a human ROBO gene disrupt hindbrain axon pathway crossing and morphogenesis.
Impact
GeneRIF
Engle et al., Los Angeles, United States. In Science, 2004
mutations in the ROBO3 gene, which shares homology with roundabout genes important in axon guidance in developing Drosophila, zebrafish, and mouse.
The divergent Robo family protein rig-1/Robo3 is a negative regulator of slit responsiveness required for midline crossing by commissural axons.
Impact
GeneRIF
Tessier-Lavigne et al., San Francisco, United States. In Cell, 2004
Expression of Rig-1 protein by commissural axons is inversely correlated with Slit sensitivity. Removal of Rig-1 results in a total failure of commissural axons to cross.
Role of Slit proteins in the vertebrate brain.
Review
Chédotal et al., Paris, France. In J Physiol Paris, 2002
Three distinct slit genes (slit1, slit2 and slit3) and three distinct robo genes (robo1, robo2 and rig-1) have been cloned in mammals.
Short-range and long-range guidance by Slit and its Robo receptors: a combinatorial code of Robo receptors controls lateral position.
Impact
Goodman et al., Berkeley, United States. In Cell, 2001
Medial axons express only Robo, intermediate axons express Robo3 and Robo, while lateral axons express Robo2, Robo3, and Robo.
Selecting a longitudinal pathway: Robo receptors specify the lateral position of axons in the Drosophila CNS.
Impact
Dickson et al., Vienna, Austria. In Cell, 2001
In this long-range function, Slit signals through the receptors Robo2 and Robo3.
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