Cho, K., Yoon, D., Yu, M., Peachey, NS and Ferreira, PA (2019) Microglial activation in an amyotrophic lateral sclerosis-like model caused by Ranbp2 loss and nucleocytoplasmic transport impairment in retinal ganglion neurons. Cell. Mol. Life Sci., 7 (17):3407-3432   (featured in News Press Release)


Ferreira, PA (2019) The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration. Cell. Mol. Life Sci., 76 (12):2247-2273.


Patil, H., Yoon, D., Bhowmick, R., Cai, Y., Cho, K. and Ferreira, PA (2019) 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. Small GTPases; 10(2):146-161.



Cho, K., Yoon, D., Qiu, S., Danziger, Z., Grill, W.M., Wetsel, W.C. and Ferreira, PA (2017) Loss of Ranbp2 in motor neurons causes the disruption of nucleocytoplasmic and chemokine signaling and proteostasis of hnRNPH3 and Mmp28, and the development of amyotrophic lateral sclerosis (ALS)-like syndromes. Dis Model Mech. 10: 559-579 - (featured in News Press Release)



Cho, K., Haney, V., Yoon, D., Hao, Y.  and Ferreira, PA (2015) Uncoupling phototoxicity-elicited neural dysmorphology and death by insidious function and selective impairment of Ran-binding protein 2 (Ranbp2), FEBS Letters, 589, 3959-3968 (PMCID: PMC4751886).


Cho, K., Orry, A., Park, SE and Ferreira, PA (2015) Targeting the Cyclophilin Domain of Ran-binding Protein 2 (Ranbp2) with Novel Small Molecules to Control the Proteostasis of STAT3, hnRNPA2B1 and M-Opsin. ACS Chemical Neuroscience 6, 1476-85 (PMCID: PMC4556350)



Patil, H., Saha, A., Senda, E., Cho, K., Haque, E., Yu, M., Qiu, S., Yoon, D., Hao, Y., Peachey, N. and Ferreira, PA (2014) 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. J. Biol. Chem. 289, 29767-29789.


Cho, K, Patil, H, Senda, E, Wang, J, Yi, H, Qiu, S, Yoon, D, Yu, M., Orry, A, Peachey, NS and Ferreira, PA (2014) Differential Loss of Prolyl-Isomerase or Chaperone Activity of Ran-binding protein 2 (Ranbp2) Unveils Distinct Physiological Roles of Its Cyclophilin Domain in Proteostasis, J. Biol. Chem. 289, 4600-4625 (PMCID: PMC3931022).



Cho K., Haque, M.*, Wang, J*, Yu, M., Hao, Y., Qiu, S., Indulekha, C., Peachey, N. and Ferreira, PA (2013) Distinct and atypical intrinsic and extrinsic cell death pathways between photoreceptor cell types upon specific ablation of Ranbp2 in cone photoreceptors, PloS Genetics, 9(6): e1003555 (PMCID: PMC3688534)


Patil, H., Cho, K., Lee, J., Yang, Y., Orry, A. and Ferreira, P.A. (2013) Kinesin-1 and mitochondrial motility control by discrimination of structurally equivalent but distinct  subdomains in Ran-GTP-binding domains of RanBP2, Open Biology, 3:120183 (PMCID: PMC3718338).



Cho, K., Webb, M., Searle, K., Yi, H. and Ferreira, PA (2012) 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), Cell. Mol. Life Sci., 69: 3511-27 (PMCID: 3445802)


Patil, H., Tserentsoodol, N., Saha, A., Hao, Y. and Webb, M. and Ferreira, PA (2012) Selective loss of RPGRIP1-dependent ciliary targeting of NPHP4, RPGR and SDCCAG8 underlies the degeneration of photoreceptor neurons, Cell Death & Disease, 3:e355 (PMCID: 3406595).


Ferreira, P.A. and Orry, A. (2012) From Drosophila to humans: Reflections on the roles of the prolyl- isomerases and chaperones, cyclophilins, in cell function and disease. J. Neurogenetics, 26:132-43; (PMCID: PMC3668307)


Patil, H, Guruju, MR, Cho, K, Yi, H, Orry, A, Kim, H and Ferreira, PA (2012) Structural and functional  plasticity of subcellular tethering, targeting and processing of RPGRIP1 by RPGR  isoforms. Biology Open, 1, 140–160 (PMCID: PMC3507198)



Fernández-Martínez, L, Letteboer, S, Mardin, CY, Weisschuh, N, Gramer, E, Weber, B, Rautenstrauss, B, Ferreira, PA, Kruse, F, Reis, A, Roepman, R and Pasutto, F. (2011) Evidence for RPGRIP1 gene as risk factor for primary open angle glaucoma. Eur.J.Hum.Genet., 19(4):445-51 (PMCID: 3060327)



Cho KI, Yi H, Tserentsoodol N, Searle K, Ferreira PA (2010) 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. Dis Model Mech. 3, 595-604 (PMCID: 2931537)



Cho K-I, Yi H, Yeh A, Tserentsoodol N, Cuadrado L, Searle K, Hao Y, Ferreira PA. (2009) Haplo-insufficiency of RanBP2 is neuroprotective against light-elicited and age-dependent degeneration of photoreceptor neurons. Cell Death Differen, 16:287-97 (PMCID: 2626153)


Cho K-I, Yi H, Desai R, Hand AR, Haas AL, Ferreira PA. (2009) The Ran-binding protein-2 (RanBP2) is an allosteric activator of the conventional kinesin-1 motor activity, KIF5B, in a minimal cell-free system.  EMBO reports, 10:480-6 (PMCID: 2680871)


Walsh, MM, Yi, H., Friedman, J., Cho, K., Tserentsoodol, N., McKinnon, S., Searle, K., Yeh, A. and Ferreira, PA. (2009) Gene and Protein Expression Pilot Profiling and Biomarkers in an Experimental Mouse Model of Hypertensive Glaucoma. Exp. Biol. Med., 234:918-30. (PMID: 19491369)


Won J, Gifford E, Smith RS, Yi H, Ferreira PA, Li T, Naggert JK, Nishina PM. (2009) RPGRIP1 is essential for normal rod photoreceptor outer segment elaboration and morphogenesis. Hum Mol Genet. 18:4329-4339 (PMCID: 2766293)



Moore D F, Gelderman M P, Ferreira PA, Fuhrmann S R, Yi H, Elkahloun A, Lix L M, Brady RO, Schiffmann R, Goldin E. (2007) Genomic abnormalities of the murine model of Fabry disease after  disease-related perturbation, a systems biology approach. Proc Natl Acad Sci, USA 104:8065-70 (PMCID: 1859990)


Cho K-I, Cai Y, Yi H, Yeh A, Aslanukov A, Ferreira PA.  (2007)  Association of the kinesin-binding domain of RanBP2 to KIF5B and KIF5C determines mitochondria localization and function. Traffic, 8:1722-35 (PMID # 17887960)


Yi H, Friedman J, Ferreira PA. (2007) The cyclophilin-like domain of RanBP2 modulates selectively the activity of the ubiquitin-proteasome system and protein biogenesis. J Biol Chem, 282:34770-8 (PMID # 17911097)



Aslanukov A, Bhowmick R, Guruju M, Oswald J, Raz D, Bush R, Sieving P, Lu X, Bock, C. Ferreira PA. (2006) RanBP2 modulates Cox11 and hexokinase I activities and haploinsufficiency of RanBP2 causes deficits in hexokinase I and glucose metabolism. PLoS Genetics, 2(10):1653-65 (PMCID: 1626108)



Lu X, Ferreira PA. (2005) Identification of novel murine- and human-specific RPGRIP1 splice-variants with  distinct expression profiles and subcellular localization. Invest Ophthalmol Vis  Sci, 46:1882-90 (PMCID: 1769349)


Lu X, Guruju M, Oswald J, Ferreira PA. (2005) Limited proteolysis differentially modulates the stability and subcellular localization of domains of RPGRIP1 that are distinctly affected by mutations in Leber's congenital amaurosis. Hum Mol Genet, 14:1327-40 (PMCID: 1769350)


Ferreira, PA.  (2005) Insights into X-linked RP3, allied diseases and underlying pathomechanisms.  Hum Mol Genet, 14(2):R259-67 (PMCID: 1769351)


Roepman R, Letteboer S, Arts HH, C. van Beersum S, Lu X, Krieger E, Ferreira PA, Cremers FM. (2005) Interaction of nephrocystin-4 and RPGRIP1 is disrupted by nephronophthisis or Leber congenital amaurosis (LCA)-associated mutations. Proc Natl Acad Sci, 102;18520-5 (PMCID: 1317916)


PubMed Search additional publications.

Duke University Medical Center, 2351 Erwin Road, DUEC 3802, Durham, NC 27710, USA