Scholars@Duke
Paulo Alexandre Ferreira

Paulo Alexandre Ferreira

Associate Professor in Ophthalmology
Ophthalmology, Vitreoretinal Diseases & Surgery
paulo.ferreira@duke.edu
Duec 3802, 2351 Erwin Road, Durham 27710
2351 Erwin Road, Duec 3802, Aeri Bldg, Room 5002, Durham 27710

Selected Publications

Nucleocytoplasmic transport at the crossroads of proteostasis, neurodegeneration and neuroprotection.

Journal Article FEBS Lett · October 2023 Featured Publication Nucleocytoplasmic transport comprises the multistep assembly, transport, and disassembly of protein and RNA cargoes entering and exiting nuclear pores. Accruing evidence supports that impairments to nucleocytoplasmic transport are a hallmark of neurodegene ... Full text Link to item Cite

Microglial activation in an amyotrophic lateral sclerosis-like model caused by Ranbp2 loss and nucleocytoplasmic transport impairment in retinal ganglion neurons.

Journal Article Cellular and molecular life sciences : CMLS · September 2019 Featured Publication Nucleocytoplasmic transport is dysregulated in sporadic and familial amyotrophic lateral sclerosis (ALS) and retinal ganglion neurons (RGNs) are purportedly involved in ALS. The Ran-binding protein 2 (Ranbp2) controls rate-limiting steps of nucleocytoplasm ... Full text Cite

The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration.

Journal Article Cell Mol Life Sci · June 2019 Featured Publication The nuclear pore is the gatekeeper of nucleocytoplasmic transport and signaling through which a vast flux of information is continuously exchanged between the nuclear and cytoplasmic compartments to maintain cellular homeostasis. A unifying and organizing ... Full text Link to item Cite

Correction to: The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration.

Journal Article Cell Mol Life Sci · June 2019 The original version of this article unfortunately contained the following misspelling and formatting mistakes. ... Full text Link to item Cite

Impairments in age-dependent ubiquitin proteostasis and structural integrity of selective neurons by uncoupling Ran GTPase from the Ran-binding domain 3 of Ranbp2 and identification of novel mitochondrial isoforms of ubiquitin-conjugating enzyme E2I (ubc9) and Ranbp2.

Journal Article Small GTPases · March 2019 Featured Publication The Ran-binding protein 2 (Ranbp2/Nup358) is a cytoplasmic and peripheral nucleoporin comprised of 4 Ran-GTP-binding domains (RBDs) that are interspersed among diverse structural domains with multifunctional activities. Our prior studies found that the RBD ... Full text Link to item Cite

Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes.

Journal Article Dis Model Mech · May 1, 2017 Featured Publication The pathogenic drivers of sporadic and familial motor neuron disease (MND), such amyotrophic lateral sclerosis (ALS), are unknown. MND impairs the Ran GTPase cycle, which controls nucleocytoplasmic transport, ribostasis and proteostasis; however, cause-eff ... Full text Link to item Cite

Uncoupling phototoxicity-elicited neural dysmorphology and death by insidious function and selective impairment of Ran-binding protein 2 (Ranbp2).

Journal Article FEBS Lett · December 21, 2015 Featured Publication Morphological disintegration of neurons is coupled invariably to neural death. In particular, disruption of outer segments of photoreceptor neurons triggers photoreceptor death regardless of the pathological stressors. We show that Ranbp2(-/-)::Tg-Ranbp2(C ... Full text Link to item Cite

Targeting the cyclophilin domain of Ran-binding protein 2 (Ranbp2) with novel small molecules to control the proteostasis of STAT3, hnRNPA2B1 and M-opsin.

Journal Article ACS Chem Neurosci · August 19, 2015 Featured Publication Cyclophilins are peptidyl cis-trans prolyl isomerases (PPIases), whose activity is typically inhibited by cyclosporine A (CsA), a potent immunosuppressor. Cyclophilins are also chaperones. Emerging evidence supports that cyclophilins present nonoverlapping ... Full text Link to item Cite

Selective impairment of a subset of Ran-GTP-binding domains of ran-binding protein 2 (Ranbp2) suffices to recapitulate the degeneration of the retinal pigment epithelium (RPE) triggered by Ranbp2 ablation.

Journal Article J Biol Chem · October 24, 2014 Featured Publication Retinal pigment epithelium (RPE) degeneration underpins diseases triggered by disparate genetic lesions, noxious insults, or both. The pleiotropic Ranbp2 controls the expression of intrinsic and extrinsic pathological stressors impinging on cellular viabil ... Full text Link to item Cite

Differential loss of prolyl isomerase or chaperone activity of Ran-binding protein 2 (Ranbp2) unveils distinct physiological roles of its cyclophilin domain in proteostasis.

Journal Article J Biol Chem · February 21, 2014 Featured Publication The immunophilins, cyclophilins, catalyze peptidyl cis-trans prolyl-isomerization (PPIase), a rate-limiting step in protein folding and a conformational switch in protein function. Cyclophilins are also chaperones. Noncatalytic mutations affecting the only ... Full text Link to item Cite

Distinct and atypical intrinsic and extrinsic cell death pathways between photoreceptor cell types upon specific ablation of Ranbp2 in cone photoreceptors.

Journal Article PLoS Genet · June 2013 Featured Publication Non-autonomous cell-death is a cardinal feature of the disintegration of neural networks in neurodegenerative diseases, but the molecular bases of this process are poorly understood. The neural retina comprises a mosaic of rod and cone photoreceptors. Cone ... Full text Open Access Link to item Cite

Kinesin-1 and mitochondrial motility control by discrimination of structurally equivalent but distinct subdomains in Ran-GTP-binding domains of Ran-binding protein 2.

Journal Article Open Biol · March 27, 2013 Featured Publication The pleckstrin homology (PH) domain is a versatile fold that mediates a variety of protein-protein and protein-phosphatidylinositol lipid interactions. The Ran-binding protein 2 (RanBP2) contains four interspersed Ran GTPase-binding domains (RBD(n = 1-4)) ... Full text Open Access Link to item Cite

Ranbp2 haploinsufficiency mediates distinct cellular and biochemical phenotypes in brain and retinal dopaminergic and glia cells elicited by the Parkinsonian neurotoxin, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).

Journal Article Cell Mol Life Sci · October 2012 Featured Publication Many components and pathways transducing multifaceted and deleterious effects of stress stimuli remain ill-defined. The Ran-binding protein 2 (RanBP2) interactome modulates the expression of a range of clinical and cell-context-dependent manifestations upo ... Full text Open Access Link to item Cite

Selective loss of RPGRIP1-dependent ciliary targeting of NPHP4, RPGR and SDCCAG8 underlies the degeneration of photoreceptor neurons.

Journal Article Cell Death Dis · July 19, 2012 Featured Publication The retinitis pigmentosa GTPase regulator (RPGR) and nephrocystin-4 (NPHP4) comprise two key partners of the assembly complex of the RPGR-interacting protein 1 (RPGRIP1). Mutations in RPGR and NPHP4 are linked to severe multisystemic diseases with strong r ... Full text Open Access Link to item Cite

From Drosophila to humans: reflections on the roles of the prolyl isomerases and chaperones, cyclophilins, in cell function and disease.

Journal Article J Neurogenet · June 2012 Featured Publication Despite remarkable advances in human genetics and other genetic model systems, the fruit fly, Drosophila melanogaster, remains a powerful experimental tool to probe with ease the inner workings of a myriad of biological and pathological processes, even whe ... Full text Link to item Cite

Structural and functional plasticity of subcellular tethering, targeting and processing of RPGRIP1 by RPGR isoforms.

Journal Article Biol Open · February 15, 2012 Featured Publication Mutations affecting the retinitis pigmentosa GTPase regulator-interacting protein 1 (RPGRIP1) interactome cause syndromic retinal dystrophies. RPGRIP1 interacts with the retinitis pigmentosa GTPase regulator (RPGR) through a domain homologous to RCC1 (RHD) ... Full text Open Access Link to item Cite

Nucleocytoplasmic transport at the crossroads of proteostasis, neurodegeneration and neuroprotection.

Journal Article FEBS Lett · October 2023 Featured Publication Nucleocytoplasmic transport comprises the multistep assembly, transport, and disassembly of protein and RNA cargoes entering and exiting nuclear pores. Accruing evidence supports that impairments to nucleocytoplasmic transport are a hallmark of neurodegene ... Full text Link to item Cite

Microglial activation in an amyotrophic lateral sclerosis-like model caused by Ranbp2 loss and nucleocytoplasmic transport impairment in retinal ganglion neurons.

Journal Article Cellular and molecular life sciences : CMLS · September 2019 Featured Publication Nucleocytoplasmic transport is dysregulated in sporadic and familial amyotrophic lateral sclerosis (ALS) and retinal ganglion neurons (RGNs) are purportedly involved in ALS. The Ran-binding protein 2 (Ranbp2) controls rate-limiting steps of nucleocytoplasm ... Full text Cite

The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration.

Journal Article Cell Mol Life Sci · June 2019 Featured Publication The nuclear pore is the gatekeeper of nucleocytoplasmic transport and signaling through which a vast flux of information is continuously exchanged between the nuclear and cytoplasmic compartments to maintain cellular homeostasis. A unifying and organizing ... Full text Link to item Cite

Correction to: The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration.

Journal Article Cell Mol Life Sci · June 2019 The original version of this article unfortunately contained the following misspelling and formatting mistakes. ... Full text Link to item Cite

Impairments in age-dependent ubiquitin proteostasis and structural integrity of selective neurons by uncoupling Ran GTPase from the Ran-binding domain 3 of Ranbp2 and identification of novel mitochondrial isoforms of ubiquitin-conjugating enzyme E2I (ubc9) and Ranbp2.

Journal Article Small GTPases · March 2019 Featured Publication The Ran-binding protein 2 (Ranbp2/Nup358) is a cytoplasmic and peripheral nucleoporin comprised of 4 Ran-GTP-binding domains (RBDs) that are interspersed among diverse structural domains with multifunctional activities. Our prior studies found that the RBD ... Full text Link to item Cite

Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes.

Journal Article Dis Model Mech · May 1, 2017 Featured Publication The pathogenic drivers of sporadic and familial motor neuron disease (MND), such amyotrophic lateral sclerosis (ALS), are unknown. MND impairs the Ran GTPase cycle, which controls nucleocytoplasmic transport, ribostasis and proteostasis; however, cause-eff ... Full text Link to item Cite

Uncoupling phototoxicity-elicited neural dysmorphology and death by insidious function and selective impairment of Ran-binding protein 2 (Ranbp2).

Journal Article FEBS Lett · December 21, 2015 Featured Publication Morphological disintegration of neurons is coupled invariably to neural death. In particular, disruption of outer segments of photoreceptor neurons triggers photoreceptor death regardless of the pathological stressors. We show that Ranbp2(-/-)::Tg-Ranbp2(C ... Full text Link to item Cite

Targeting the cyclophilin domain of Ran-binding protein 2 (Ranbp2) with novel small molecules to control the proteostasis of STAT3, hnRNPA2B1 and M-opsin.

Journal Article ACS Chem Neurosci · August 19, 2015 Featured Publication Cyclophilins are peptidyl cis-trans prolyl isomerases (PPIases), whose activity is typically inhibited by cyclosporine A (CsA), a potent immunosuppressor. Cyclophilins are also chaperones. Emerging evidence supports that cyclophilins present nonoverlapping ... Full text Link to item Cite

Selective impairment of a subset of Ran-GTP-binding domains of ran-binding protein 2 (Ranbp2) suffices to recapitulate the degeneration of the retinal pigment epithelium (RPE) triggered by Ranbp2 ablation.

Journal Article J Biol Chem · October 24, 2014 Featured Publication Retinal pigment epithelium (RPE) degeneration underpins diseases triggered by disparate genetic lesions, noxious insults, or both. The pleiotropic Ranbp2 controls the expression of intrinsic and extrinsic pathological stressors impinging on cellular viabil ... Full text Link to item Cite

Differential loss of prolyl isomerase or chaperone activity of Ran-binding protein 2 (Ranbp2) unveils distinct physiological roles of its cyclophilin domain in proteostasis.

Journal Article J Biol Chem · February 21, 2014 Featured Publication The immunophilins, cyclophilins, catalyze peptidyl cis-trans prolyl-isomerization (PPIase), a rate-limiting step in protein folding and a conformational switch in protein function. Cyclophilins are also chaperones. Noncatalytic mutations affecting the only ... Full text Link to item Cite

Distinct and atypical intrinsic and extrinsic cell death pathways between photoreceptor cell types upon specific ablation of Ranbp2 in cone photoreceptors.

Journal Article PLoS Genet · June 2013 Featured Publication Non-autonomous cell-death is a cardinal feature of the disintegration of neural networks in neurodegenerative diseases, but the molecular bases of this process are poorly understood. The neural retina comprises a mosaic of rod and cone photoreceptors. Cone ... Full text Open Access Link to item Cite

Kinesin-1 and mitochondrial motility control by discrimination of structurally equivalent but distinct subdomains in Ran-GTP-binding domains of Ran-binding protein 2.

Journal Article Open Biol · March 27, 2013 Featured Publication The pleckstrin homology (PH) domain is a versatile fold that mediates a variety of protein-protein and protein-phosphatidylinositol lipid interactions. The Ran-binding protein 2 (RanBP2) contains four interspersed Ran GTPase-binding domains (RBD(n = 1-4)) ... Full text Open Access Link to item Cite

Ranbp2 haploinsufficiency mediates distinct cellular and biochemical phenotypes in brain and retinal dopaminergic and glia cells elicited by the Parkinsonian neurotoxin, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).

Journal Article Cell Mol Life Sci · October 2012 Featured Publication Many components and pathways transducing multifaceted and deleterious effects of stress stimuli remain ill-defined. The Ran-binding protein 2 (RanBP2) interactome modulates the expression of a range of clinical and cell-context-dependent manifestations upo ... Full text Open Access Link to item Cite

Selective loss of RPGRIP1-dependent ciliary targeting of NPHP4, RPGR and SDCCAG8 underlies the degeneration of photoreceptor neurons.

Journal Article Cell Death Dis · July 19, 2012 Featured Publication The retinitis pigmentosa GTPase regulator (RPGR) and nephrocystin-4 (NPHP4) comprise two key partners of the assembly complex of the RPGR-interacting protein 1 (RPGRIP1). Mutations in RPGR and NPHP4 are linked to severe multisystemic diseases with strong r ... Full text Open Access Link to item Cite

From Drosophila to humans: reflections on the roles of the prolyl isomerases and chaperones, cyclophilins, in cell function and disease.

Journal Article J Neurogenet · June 2012 Featured Publication Despite remarkable advances in human genetics and other genetic model systems, the fruit fly, Drosophila melanogaster, remains a powerful experimental tool to probe with ease the inner workings of a myriad of biological and pathological processes, even whe ... Full text Link to item Cite

Structural and functional plasticity of subcellular tethering, targeting and processing of RPGRIP1 by RPGR isoforms.

Journal Article Biol Open · February 15, 2012 Featured Publication Mutations affecting the retinitis pigmentosa GTPase regulator-interacting protein 1 (RPGRIP1) interactome cause syndromic retinal dystrophies. RPGRIP1 interacts with the retinitis pigmentosa GTPase regulator (RPGR) through a domain homologous to RCC1 (RHD) ... Full text Open Access Link to item Cite

Evidence for RPGRIP1 gene as risk factor for primary open angle glaucoma.

Journal Article Eur J Hum Genet · April 2011 Featured Publication Glaucoma is a genetically heterogeneous disorder and is the second cause of blindness worldwide owing to the progressive degeneration of retinal ganglion neurons. Very few genes causing glaucoma were identified to this date. In this study, we screened 10 c ... Full text Link to item Cite

Ageing of the units of biofiltration of waste water: Balance assessment after 10 years of functioning

Journal Article Eau, l'INDUSTRIE, les Nuisances · February 1, 2011 For more than 10 years, the Interdepartmental Association for Sewage disposal in Paris Conurbation (SIAAP), which is in charge of both the transport and the treatment of the Great Paris wastewaters, use biofiltres as a step of the water treatment in its pl ... Cite

Neuroprotection resulting from insufficiency of RANBP2 is associated with the modulation of protein and lipid homeostasis of functionally diverse but linked pathways in response to oxidative stress.

Journal Article Dis Model Mech · 2010 Featured Publication Oxidative stress is a deleterious stressor associated with a plethora of disease and aging manifestations, including neurodegenerative disorders, yet very few factors and mechanisms promoting the neuroprotection of photoreceptor and other neurons against o ... Full text Open Access Link to item Cite

RPGRIP1 is essential for normal rod photoreceptor outer segment elaboration and morphogenesis.

Journal Article Hum Mol Genet · November 15, 2009 Featured Publication The function of the retinitis pigmentosa GTPase regulator interacting protein 1 (RPGRIP1) gene is currently not known. However, mutations within the gene lead to Leber Congenital Amaurosis and autosomal recessive retinitis pigmentosa in human patients. In ... Full text Link to item Cite

Gene and protein expression pilot profiling and biomarkers in an experimental mouse model of hypertensive glaucoma.

Journal Article Exp Biol Med (Maywood) · August 2009 Featured Publication Glaucoma is a group of genetically heterogeneous neurodegenerative disorders causing the degeneration of the ganglion neurons of the retina. Increased intraocular pressure (IOP) is a hallmark risk factor promoting the death of ganglion neurons of the retin ... Full text Link to item Cite

RANBP2 is an allosteric activator of the conventional kinesin-1 motor protein, KIF5B, in a minimal cell-free system.

Journal Article EMBO Rep · May 2009 Featured Publication The association of cargoes to kinesins is thought to promote kinesin activation, yet the validation of such a model with native cargoes is lacking because none is known to activate kinesins directly in an in vitro system of purified components. The RAN-bin ... Full text Link to item Cite

Haploinsufficiency of RanBP2 is neuroprotective against light-elicited and age-dependent degeneration of photoreceptor neurons.

Journal Article Cell Death Differ · February 2009 Featured Publication Prolonged light exposure is a determinant factor in inducing neurodegeneration of photoreceptors by apoptosis. Yet, the molecular bases of the pathways and components triggering this cell death event are elusive. Here, we reveal a prominent age-dependent i ... Full text Link to item Cite

Association of the kinesin-binding domain of RanBP2 to KIF5B and KIF5C determines mitochondria localization and function.

Journal Article Traffic · December 2007 Featured Publication The Ran-binding protein 2 (RanBP2) is a large mosaic protein with a pleiotropic role in cell function. Although the contribution of each partner and domain of RanBP2 to its biological functions are not understood, physiological deficits of RanBP2 downregul ... Full text Link to item Cite

The cyclophilin-like domain of Ran-binding protein-2 modulates selectively the activity of the ubiquitin-proteasome system and protein biogenesis.

Journal Article J Biol Chem · November 30, 2007 Featured Publication The ubiquitin-proteasome system (UPS) plays a critical role in protein degradation. The 19S regulatory particle (RP) of the 26S proteasome mediates the recognition, deubiquitylation, unfolding, and channeling of ubiquitylated substrates to the 20S proteaso ... Full text Link to item Cite

Genomic abnormalities of the murine model of Fabry disease after disease-related perturbation, a systems biology approach.

Journal Article Proc Natl Acad Sci U S A · May 8, 2007 Featured Publication Fabry disease is a disorder of alpha-D-galactosyl-containing glycolipids resulting from a deficiency of alpha-galactosidase A. Patients have a poorly understood vascular dysregulation. We hypothesized that disease-related perturbation by using enzyme repla ... Full text Link to item Cite

RanBP2 modulates Cox11 and hexokinase I activities and haploinsufficiency of RanBP2 causes deficits in glucose metabolism.

Journal Article PLoS Genet · October 2006 Featured Publication The Ran-binding protein 2 (RanBP2) is a large multimodular and pleiotropic protein. Several molecular partners with distinct functions interacting specifically with selective modules of RanBP2 have been identified. Yet, the significance of these interactio ... Full text Open Access Link to item Cite

Interaction of nephrocystin-4 and RPGRIP1 is disrupted by nephronophthisis or Leber congenital amaurosis-associated mutations.

Journal Article Proc Natl Acad Sci U S A · December 20, 2005 Featured Publication RPGR-interacting protein 1 (RPGRIP1) is a key component of cone and rod photoreceptor cells, where it interacts with RPGR (retinitis pigmentosa GTPase regulator). Mutations in RPGRIP1 lead to autosomal recessive congenital blindness [Leber congenital amaur ... Full text Link to item Cite

Insights into X-linked retinitis pigmentosa type 3, allied diseases and underlying pathomechanisms.

Journal Article Hum Mol Genet · October 15, 2005 Featured Publication In the past decade, we have witnessed great advances in the identification of genes underlying numerous neurodegenerative diseases and the stark complexity determining genotype-phenotype relationships that lead to the impairment, and ultimately, premature ... Full text Link to item Cite

Identification of novel murine- and human-specific RPGRIP1 splice variants with distinct expression profiles and subcellular localization.

Journal Article Invest Ophthalmol Vis Sci · June 2005 Featured Publication PURPOSE: Mutations in RPGRIP1 cause Leber congenital amaurosis. The human and bovine RPGRIP1 undergo alternative splicing. A single murine rpgrip1 transcript has been reported, but distinct expression profiles of RPGRIP1 isoforms exist between species in t ... Full text Link to item Cite

Limited proteolysis differentially modulates the stability and subcellular localization of domains of RPGRIP1 that are distinctly affected by mutations in Leber's congenital amaurosis.

Journal Article Hum Mol Genet · May 15, 2005 Featured Publication The retinitis pigmentosa GTPase regulator (RPGR) protein interacts with the retinitis pigmentosa GTPase regulator interacting protein-1 (RPGRIP1). Genetic lesions in the cognate genes lead to distinct and severe human retinal dystrophies. The biological ro ... Full text Link to item Cite

Community - Acquired pneumonia

Conference Nascer e Crescer · December 1, 2004 Cite

RPGRIP1s with distinct neuronal localization and biochemical properties associate selectively with RanBP2 in amacrine neurons.

Journal Article Hum Mol Genet · August 1, 2003 Featured Publication RPGR and RPGRIP1 are molecular partners with vital roles in retinal function. Mutations in RPGR are implicated in heterogeneous retinal phenotypes, while those in RPGRIP1 lead to Leber congenital amaurosis. RPGR and RPGRIP1s differentially localize in phot ... Full text Link to item Cite

Identification of RanBP2- and kinesin-mediated transport pathways with restricted neuronal and subcellular localization.

Journal Article Traffic · September 2002 Featured Publication Ran-binding proteins, karyopherins, and RanGTPase mediate and impart directionality to nucleocytoplasmic transport processes. This biological process remains elusive in neurons. RanBP2 has been localized at the nuclear pore complexes and is very abundant i ... Full text Link to item Cite

Species-specific subcellular localization of RPGR and RPGRIP isoforms: implications for the phenotypic variability of congenital retinopathies among species.

Journal Article Hum Mol Genet · August 1, 2002 Featured Publication The retinitis pigmentosa GTPase regulator (RPGR) is encoded by the X-linked RP3 locus, which upon genetic lesions leads to neurodegeneration of photoreceptors and blindness. The findings that RPGR specifically and directly interacts in vivo and in vitro wi ... Full text Link to item Cite

[Other indications for surfactant].

Journal Article An Esp Pediatr · January 2002 OBJECTIVE: The introduction of surfactant replacement therapy in the management of respiratory distress syndrome in the premature infant was a remarkable advance in neonatal intensive care. In the last few years, recognition of the role played by surfactan ... Link to item Cite

The docking of kinesins, KIF5B and KIF5C, to Ran-binding protein 2 (RanBP2) is mediated via a novel RanBP2 domain.

Journal Article J Biol Chem · November 9, 2001 Featured Publication The Ran-binding protein 2 (RanBP2) is a vertebrate mosaic protein composed of four interspersed RanGTPase binding domains (RBDs), a variable and species-specific zinc finger cluster domain, leucine-rich, cyclophilin, and cyclophilin-like (CLD) domains. Fun ... Full text Link to item Cite

Genomic organization, expression, and localization of murine Ran-binding protein 2 (RanBP2) gene.

Journal Article Mamm Genome · June 2001 Featured Publication The Ran-binding protein 2 (RanBP2) is a giant scaffold and mosaic cyclophilin-related nucleoporin implicated in the Ran-GTPase cycle. There are no orthologs of the RanBP2 gene in yeast and Drosophila genomes. In humans, this bona fide gene is partially dup ... Full text Link to item Cite

Acute methadone intoxication - case - report

Journal Article Nascer e Crescer · May 12, 2001 The authors describe a clinical report of methadone intoxication in a four year old child, whose parents were in a program of opioid substitution. A revision on opioidintoxication in children is made, giving attention to the efficacy of precocious specific ... Cite

The retinitis pigmentosa GTPase regulator (RPGR) interacts with novel transport-like proteins in the outer segments of rod photoreceptors.

Journal Article Hum Mol Genet · September 1, 2000 Featured Publication Mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene cause X-linked retinitis pigmentosa type 3 (RP3), a severe, progressive and degenerative retinal dystrophy eventually leading to complete blindness. RPGR is ubiquitously expressed, yet muta ... Full text Link to item Cite

The zinc finger cluster domain of RanBP2 is a specific docking site for the nuclear export factor, exportin-1.

Journal Article J Biol Chem · December 24, 1999 Featured Publication The Ran-binding protein 2 (RanBP2) is a large scaffold cyclophilin-related protein expressed in photoreceptor cells. Red/green opsin, Ran-GTPase, and the 19 S regulatory complex of the proteasome associate with specific RanBP2 structural modules. Some of t ... Full text Link to item Cite

The cyclophilin-like domain mediates the association of Ran-binding protein 2 with subunits of the 19 S regulatory complex of the proteasome.

Journal Article J Biol Chem · September 18, 1998 Featured Publication The combination of the Ran-binding domain 4 and cyclophilin domains of Ran-binding protein 2 selectively associate with a subset of G protein-coupled receptors, red/green opsins, upon cis-trans prolyl isomerase-dependent and direct modification of opsin fo ... Full text Link to item Cite

Design and marketing innovation

Journal Article Journal of the Textile Institute · January 1, 1998 Trends in global textile and clothing strategies are briefly reviewed to introduce the Portuguese context, and the development of Design & Marketing education and research at The University of Minho. The development of textile-oriented multimedia technolog ... Full text Cite

Interconversion of red opsin isoforms by the cyclophilin-related chaperone protein Ran-binding protein 2.

Journal Article Proc Natl Acad Sci U S A · February 18, 1997 Featured Publication Ran-binding protein 2 (RanBP2) (type II) is a retinal cyclophilin-related protein that binds Ran-GTPase. Type I cyclophilin is a shorter, alternatively spliced isoform of RanBP2. Recently, we showed that the Ran-binding domain 4 (RBD4)/cyclophilin (CY) sup ... Full text Link to item Cite

Cyclophilin-related protein RanBP2 acts as chaperone for red/green opsin.

Journal Article Nature · October 17, 1996 Featured Publication Cyclophilins are ubiquitous and abundant proteins that exhibit peptidyl prolyl cis-trans isomerization (PPlase) activity in vitro. Their functions in vivo, however, are not well understood. Two new retinal cyclophilin isoforms, types I and II, are highly e ... Full text Link to item Cite

Retina-specifically expressed novel subtypes of bovine cyclophilin.

Journal Article J Biol Chem · September 29, 1995 Featured Publication The Drosophila ninaA gene encodes photoreceptor-specific cyclophilin thought to play a critical role in rhodopsin folding or transport during its synthesis or maturation in the most abundant subclass of photoreceptors. Cyclophilins comprise a highly conser ... Full text Link to item Cite

Bovine phospholipase C highly homologous to the norpA protein of Drosophila is expressed specifically in cones.

Journal Article J Biol Chem · February 4, 1994 Featured Publication The Drosophila norpA gene encodes a phosphatidylinositol-specific phospholipase C (PI-PLC) expressed predominantly in photoreceptors and involved in phototransduction. However, no direct role for a phospholipase C in vertebrate phototransduction has been i ... Link to item Cite

Distinctive subtypes of bovine phospholipase C that have preferential expression in the retina and high homology to the norpA gene product of Drosophila.

Journal Article Proc Natl Acad Sci U S A · July 1, 1993 Featured Publication The Drosophila norpA gene encodes a phospholipase C involved in phototransduction. However, phospholipase C apparently is not directly involved in phototransduction in vertebrate photoreceptors, although light-activated phospholipase C activity has been re ... Full text Link to item Cite