Research Topics
Genomes and Genes | Surafel MulugetaSummaryAffiliation: University of Pennsylvania Country: USA Publications
Research Grants
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Publications
A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signalingMarkus Woischnik
Department of Pneumology, Dr von Hauner Children s Hospital, Ludwig Maximilians University, Lindwurmstr 4, Munich 80337, Germany
BMC Cell Biol 11:88. 2010..The most frequent SFTPC mutation in ILD patients leads to a threonine for isoleucine substitution at position 73 (I73T) of the SP-C preprotein (proSP-C), however little is known about the cellular consequences of SP-CI73T expression...
Surfactant protein C: its unique properties and emerging immunomodulatory role in the lungSurafel Mulugeta
Pulmonary and Critical Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4318, USA
Microbes Infect 8:2317-23. 2006....- Processing of surfactant protein C requires a type II transmembrane topology directed by juxtamembrane positively charged residuesSurafel Mulugeta
Lung Epithelial Cell Biology Laboratories, Pulmonary and Critical Care Division, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4318, USA
J Biol Chem 278:47979-86. 2003..Moreover this study provides a likely precedent for a mechanism in disorders associated with mutations in the membrane-flanking region of integral membrane proteins... - Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanismsSurafel Mulugeta
Pulmonary, Allergy, and Critical Care Division, Univ of Pennsylvania School of Medicine, Vernon and Shirley Hill Pavilion, Suite H418, 380 South Univ Ave, Philadelphia, PA 19104, USA
Am J Physiol Lung Cell Mol Physiol 293:L720-9. 2007..These results suggest common cellular responses, including initiation of cell-death signaling pathways, to these lung disease-associated BRISPC proteins... - A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activationSurafel Mulugeta
Pulmonary and Critical Care Division, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6160, USA
Am J Respir Cell Mol Biol 32:521-30. 2005.... - A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recyclingMichael F Beers
Pulmonary, Allergy, and Critical Care Division, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA
Traffic 12:1196-210. 2011..Our data provide evidence for a novel cellular mechanism for conformational protein-associated diseases and suggest a paradigm for mistargeted proteins involved in the disruption of the endosomal/lysosomal sorting machinery... - Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C genePaul A Stevens
Pulmonary and Critical Care Division, University of Pennsylvania School of Medicine, 421 Curie Blvd, Philadelphia, PA 19104-6160, USA
Pediatr Res 57:89-98. 2005..We propose that, similar to other storage disorders, lung injury results from induction of a toxic gain of function induced by the mutant product that is subject to genetic modifiers and environmental influences... - Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negativeWen-Jing Wang
Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
J Cell Sci 116:683-92. 2003..The heterotypic oligomerization of hSP-C(1-197) and hSP-C(deltaExon4) provides a molecular mechanism for the dominant-negative effect observed in vivo... - Anterograde transport of surfactant protein C proprotein to distal processing compartments requires PPDY-mediated association with Nedd4 ubiquitin ligasesAdam Kotorashvili
Surfactant Biology Laboratories, Department of Medicine, University of Pennsylvania School of Medicine, and Department of Pediatrics, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
J Biol Chem 284:16667-78. 2009..We speculate that the Nedd4/proSP-C tandem is part of a larger protein complex containing a ubiquitinated component that further directs its transport... - A novel conserved targeting motif found in ABCA transporters mediates trafficking to early post-Golgi compartmentsMichael F Beers
Department of Medicine, Pulmonary, Allergy, and Critical Care Division, University of Pennsylvania, Philadelphia, PA, USA
J Lipid Res 52:1471-82. 2011.... - Biosynthesis of surfactant protein C (SP-C). Sorting of SP-C proprotein involves homomeric association via a signal anchor domainWen Jing Wang
Lung Epithelial Cell Biology Laboratories, Pulmonary and Critical Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
J Biol Chem 277:19929-37. 2002..We speculate that heterotypic oligomerization of wild type with SP-C folding mutants produces a dominant negative thus contributing to the pathology of chronic lung disease associated with patients heterozygous for mutant SP-C alleles... - Multiple ways to die: delineation of the unfolded protein response and apoptosis induced by Surfactant Protein C BRICHOS mutantsJean Ann Maguire
Pulmonary and Critical Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4539, United States
Int J Biochem Cell Biol 44:101-12. 2012.... - Endoplasmic reticulum stress induced by surfactant protein C BRICHOS mutants promotes proinflammatory signaling by epithelial cellsJean Ann Maguire
Surfactant Biology Laboratories, Pulmonary and Critical Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, USA
Am J Respir Cell Mol Biol 44:404-14. 2011..Taken together, the results support the role of JNK signaling in mediating SP-C BRICHOS-induced cytokine release, and provide a link between SP-C BRICHOS mutants and proinflammatory cytokine signaling... - Identification of LBM180, a lamellar body limiting membrane protein of alveolar type II cells, as the ABC transporter protein ABCA3Surafel Mulugeta
Institute for Environmental Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
J Biol Chem 277:22147-55. 2002.... - Surfactant protein C biosynthesis and its emerging role in conformational lung diseaseMichael F Beers
Pulmonary and Critical Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6061, USA
Annu Rev Physiol 67:663-96. 2005....
Research Grants
- ABCA3: Biosynthesis, Trafficking, and Cellular Responses in Health and DiseaseSurafel Mulugeta; Fiscal Year: 2009..We believe the knowledge gained from this project will have a broad application not only toward a better understanding of lung pathogenesis but will offer insights for targeted therapeutic intervention. ..
- ABCA3: Biosynthesis, Trafficking, and Cellular Responses in Health and DiseaseSurafel Mulugeta; Fiscal Year: 2010..We believe the knowledge gained from this project will have a broad application not only toward a better understanding of lung pathogenesis but will offer insights for targeted therapeutic intervention. ..
- ABCA3: Biosynthesis, Trafficking, and Cellular Responses in Health and DiseaseSurafel Mulugeta; Fiscal Year: 2009..We believe the knowledge gained from this project will have a broad application not only toward a better understanding of lung pathogenesis but will offer insights for targeted therapeutic intervention. ..