Research Topics
Genomes and Genes
| Juan S BonifacinoSummaryAffiliation: National Institutes of Health Country: USA Publications
| Collaborators
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Detail Information
Publications
Stonin 2: an adaptor-like protein that interacts with components of the endocytic machineryJ A Martina
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Biol 153:1111-20. 2001..These observations suggest that stonin 2 is a novel component of the general endocytic machinery...- GGAs: a family of ADP ribosylation factor-binding proteins related to adaptors and associated with the Golgi complexE C Dell'Angelica
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Biol 149:81-94. 2000..Disruption of both GGA genes in yeast resulted in impaired trafficking of carboxypeptidase Y to the vacuole. These observations suggest that GGAs are components of ARF-regulated coats that mediate protein trafficking at the TGN... - A diacidic motif in human immunodeficiency virus type 1 Nef is a novel determinant of binding to AP-2O Wolf Lindwasser
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T, Room 101, National Institutes of Health, Bethesda, MD 20892, USA
J Virol 82:1166-74. 2008.... - RetromerJuan S Bonifacino
Cell Biology and Metabolism Program, National Institute of Child Health and Human Development, Building 18T Room 101, National Institutes of Health, Bethesda, MD 20892, USA
Curr Opin Cell Biol 20:427-36. 2008..Recent studies have implicated retromer in a broad range of physiological, developmental and pathological processes, underscoring the critical nature of retrograde transport mediated by this complex... 
Retrograde transport from endosomes to the trans-Golgi networkJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Nat Rev Mol Cell Biol 7:568-79. 2006..We also propose a central role for a 'tubular endosomal network' in sorting to recycling pathways that lead not only to the trans-Golgi network but also to different plasma-membrane domains and to specialized storage vesicles...- Signals for sorting of transmembrane proteins to endosomes and lysosomesJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Annu Rev Biochem 72:395-447. 2003..This complex array of signals and recognition proteins ensures the dynamic but accurate distribution of transmembrane proteins to different compartments of the endosomal-lysosomal system... - The GGA proteins: adaptors on the moveJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T Room 101, National Institutes of Health, Bethesda, Maryland 20892, USA
Nat Rev Mol Cell Biol 5:23-32. 2004.... - Ubiquitin and the control of protein fate in the secretory and endocytic pathwaysJ S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 5430, USA
Annu Rev Cell Dev Biol 14:19-57. 1998..These recent findings imply that ubiquitin plays more diverse roles in the regulation of the fate of cellular proteins than originally anticipated... - Transport according to GARP: receiving retrograde cargo at the trans-Golgi networkJuan S Bonifacino
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Trends Cell Biol 21:159-67. 2011..Loss of GARP function compromises the growth, fertility and/or viability of the defective organisms, emphasizing the essential nature of GARP-mediated retrograde transport... - The mechanisms of vesicle budding and fusionJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Cell 116:153-66. 2004..Precise regulation of these two aspects of vesicular transport ensures efficient cargo transfer while preserving organelle identity... - Insights into the biogenesis of lysosome-related organelles from the study of the Hermansky-Pudlak syndromeJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T Room 101, National Institutes of Health, Bethesda, MD 20892, USA
Ann N Y Acad Sci 1038:103-14. 2004..Further studies of these complexes should contribute to the elucidation of the mechanisms of LRO biogenesis and the pathogenesis of HPS... - CD4 down-regulation by HIV-1 and simian immunodeficiency virus (SIV) Nef proteins involves both internalization and intracellular retention mechanismsJeremy J Rose
Laboratory of Molecular Microbiology, NIAID, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 280:7413-26. 2005..In contrast, AP-2-mediated enhanced endocytosis constitutes the predominant mechanism for SIV (MAC-239 strain) Nef-induced down-regulation of human CD4 in human cells... - Definition of the consensus motif recognized by gamma-adaptin ear domainsRafael Mattera
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 279:8018-28. 2004..These findings shed light on the mechanism of accessory protein recruitment to trans-Golgi network and endosomal clathrin coats... - The trihelical bundle subdomain of the GGA proteins interacts with multiple partners through overlapping but distinct sitesRafael Mattera
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 279:31409-18. 2004..The different GAT binding partners engage in both competitive and cooperative interactions that may be important for the function of the GGAs in protein sorting... - Assembly of the biogenesis of lysosome-related organelles complex-3 (BLOC-3) and its interaction with Rab9Daniel P Kloer
Laboratory of Molecular Biology, NIDDK, Eunice Kennedy Shriver NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 285:7794-804. 2010..This interaction is mediated by HPS4 and the switch I and II regions of Rab9. These characteristics indicate that BLOC-3 might function as a Rab9 effector in the biogenesis of LROs... - Downregulation of CD4 by human immunodeficiency virus type 1 Nef is dependent on clathrin and involves direct interaction of Nef with the AP2 clathrin adaptorRittik Chaudhuri
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Bldg 18T, Rm 101, National Institutes of Health, Bethesda, MD 20892, USA
J Virol 81:3877-90. 2007..Together, these results support a model in which HIV-1 Nef downregulates CD4 by promoting its accelerated endocytosis by a clathrin/AP2 pathway... - Adaptor protein 2-mediated endocytosis of the β-secretase BACE1 is dispensable for amyloid precursor protein processingYogikala Prabhu
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 23:2339-51. 2012..Taken together, these observations support the notion that BACE1 is capable of cleaving APP in late compartments of the secretory pathway... - Ang2/fat-free is a conserved subunit of the Golgi-associated retrograde protein complexF Javier Pérez-Victoria
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 21:3386-95. 2010..These findings indicate that Ang2 is the missing component of the GARP complex in most eukaryotes... - Requirement of the human GARP complex for mannose 6-phosphate-receptor-dependent sorting of cathepsin D to lysosomesF Javier Pérez-Victoria
Cell Biology and Metabolism Program, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 19:2350-62. 2008..We also report that a Vps54 mutant protein in the Wobbler mouse strain is active in retrograde transport, thus explaining the viability of these mutant mice... - Structural mechanism for ubiquitinated-cargo recognition by the Golgi-localized, gamma-ear-containing, ADP-ribosylation-factor-binding proteinsGali Prag
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 102:2334-9. 2005..This ability highlights the GAT domain as a hub for interactions with multiple partners in trafficking... - The trans-Golgi network accessory protein p56 promotes long-range movement of GGA/clathrin-containing transport carriers and lysosomal enzyme sortingGonzalo A Mardones
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 18:3486-501. 2007..We conclude that p56 tightly cooperates with the GGAs in the sorting of cathepsin D to lysosomes, probably by enabling the movement of GGA-containing TCs... - Coatomer-dependent protein delivery to lipid dropletsKrishnakant G Soni
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Sci 122:1834-41. 2009..Here we demonstrate that ATGL and ADRP, but not TIP47, are delivered to LDs by a pathway mediated by the COPI and COPII coatomer proteins and their corresponding regulators... - Structural basis for ubiquitin recognition and autoubiquitination by Rabex-5Sangho Lee
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, US National Institutes of Health NIH, Bethesda, Maryland 20892, USA
Nat Struct Mol Biol 13:264-71. 2006..The A20 zinc-finger diaromatic patch mediates ubiquitin-ligase activity by directly recruiting a ubiquitin-loaded ubiquitin-conjugating enzyme... - An ear-core interaction regulates the recruitment of the AP-3 complex to membranesStephane Lefrancois
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Dev Cell 7:619-25. 2004..These observations suggest a new regulatory mechanism for the recruitment of AP-3 to membranes involving delta-ear-sigma3 interactions... - Recognition of accessory protein motifs by the gamma-adaptin ear domain of GGA3Gregory J Miller
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland 20892, USA
Nat Struct Biol 10:599-606. 2003.... - Interchangeable but essential functions of SNX1 and SNX2 in the association of retromer with endosomes and the trafficking of mannose 6-phosphate receptorsRaul Rojas
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T Room 101, National Institutes of Health, Bethesda, MD 20892, USA
Mol Cell Biol 27:1112-24. 2007.... - Phosphoregulation of sorting signal-VHS domain interactions by a direct electrostatic mechanismYukio Kato
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Nat Struct Biol 9:532-6. 2002.... - Canonical interaction of cyclin G associated kinase with adaptor protein 1 regulates lysosomal enzyme sortingSatoshi Kametaka
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, and Laboratory of Cell Biology, National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 18:2991-3001. 2007..Thus, interactions between the PsiG[PDE][PsiLM]-motif sequences in GAK and the AP1-gamma-ear domain are critical for the recruitment of GAK to the TGN and the function of GAK in lysosomal enzyme sorting... - A basic patch on alpha-adaptin is required for binding of human immunodeficiency virus type 1 Nef and cooperative assembly of a CD4-Nef-AP-2 complexRittik Chaudhuri
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Building 18T, Room 101, National Institutes of Health, Bethesda, MD 20892, USA
J Virol 83:2518-30. 2009..This cooperativity explains how Nef is able to efficiently downregulate CD4 despite weak binary interactions between components of the tripartite complex... - The Vps27/Hse1 complex is a GAT domain-based scaffold for ubiquitin-dependent sortingGali Prag
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, U S Department of Health and Human Services, Bethesda, MD 20892, USA
Dev Cell 12:973-86. 2007..Coarse-grained Monte Carlo simulations of the Vps27/Hse1 complex on a membrane show how the complex binds cooperatively to lipids and ubiquitinated membrane proteins and acts as a scaffold for ubiquitination reactions... - Sorting of the Alzheimer's disease amyloid precursor protein mediated by the AP-4 complexPatricia V Burgos
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Dev Cell 18:425-36. 2010.... - Morphology and dynamics of clathrin/GGA1-coated carriers budding from the trans-Golgi networkRosa Puertollano
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development and Laboratory of Cell Biology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
Mol Biol Cell 14:1545-57. 2003.... - Multilayered mechanism of CD4 downregulation by HIV-1 Vpu involving distinct ER retention and ERAD targeting stepsJavier G Magadán
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America
PLoS Pathog 6:e1000869. 2010..The multiple levels at which Vpu engages these cellular quality control mechanisms underscore the importance of ensuring profound suppression of CD4 to the life cycle of HIV-1... - Interactions of GGA3 with the ubiquitin sorting machineryRosa Puertollano
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Nat Cell Biol 6:244-51. 2004..The VHS and GAT domains of GGA3 are responsible for this binding, as well as for interactions with TSG101, a component of the ubiquitin-dependent sorting machinery. Thus, GGAs may have additional roles in sorting of ubiquitinated cargo... - Regulation of retromer recruitment to endosomes by sequential action of Rab5 and Rab7Raul Rojas
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Biol 183:513-26. 2008..These findings demonstrate that Rab5 and Rab7 act in concert to regulate retromer recruitment to endosomes... - Recognition of dileucine-based sorting signals from HIV-1 Nef and LIMP-II by the AP-1 gamma-sigma1 and AP-3 delta-sigma3 hemicomplexesKaty Janvier
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Biol 163:1281-90. 2003..These observations reveal a novel mode of recognition of sorting signals involving the gamma/delta and sigma subunits of AP-1 and AP-3... - Human immunodeficiency virus type 1 Nef protein targets CD4 to the multivesicular body pathwayLuis L P daSilva
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Building 18T, Room 101, National Institutes of Health, Bethesda, MD 20892, USA
J Virol 83:6578-90. 2009..We also show that both CD4 and Nef are ubiquitinated on lysine residues, but this modification is dispensable for Nef-induced targeting of CD4 to the MVB pathway... - The Rab5 guanine nucleotide exchange factor Rabex-5 binds ubiquitin (Ub) and functions as a Ub ligase through an atypical Ub-interacting motif and a zinc finger domainRafael Mattera
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892
J Biol Chem 281:6874-83. 2006..Moreover, the demonstration that Rabex-5 is a ubiquitin ligase that binds ubiquitin and undergoes ubiquitination indicates that its role in endosome fusion may be subject to additional regulation by ubiquitin-dependent modifications... - Adaptor and clathrin exchange at the plasma membrane and trans-Golgi networkXufeng Wu
Laboratory of Cell Biology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
Mol Biol Cell 14:516-28. 2003..We conclude that clathrin-coated pits are dynamic structures with rapid exchange of both clathrin and adaptors and that adaptors are able to exchange independently of clathrin when clathrin exchange is blocked... - Functional architecture of the retromer cargo-recognition complexAitor Hierro
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, US Department of Health and Human Services, Bethesda, Maryland 20892, USA
Nature 449:1063-7. 2007..This extended structure presents multiple binding sites for the SNX complex and receptor cargo, and appears capable of flexing to conform to curved vesicular membranes... - Involvement of clathrin and AP-2 in the trafficking of MHC class II molecules to antigen-processing compartmentsPeter J McCormick
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 102:7910-5. 2005..Because AP-2 is only associated with the plasma membrane, these results also indicate that a significant pool of MHC-II molecules traffic to the endosomal-lysosomal system by means of the cell surface... - Direct binding to Rsp5p regulates ubiquitination-independent vacuolar transport of Sna3pHadiya Watson
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 18:1781-9. 2007..Our data demonstrate that Sna3p follows a novel ubiquitination-independent, but Rsp5p-mediated, sorting pathway to the vacuole... - Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complexF Javier Pérez-Victoria
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 107:12860-5. 2010..Severely reduced levels of mutant Vps54 and, consequently, of the whole GARP complex underlie the phenotype of the wobbler mouse... - Role of the mammalian retromer in sorting of the cation-independent mannose 6-phosphate receptorCecilia N Arighi
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Biol 165:123-33. 2004..These observations indicate that retromer prevents the delivery of the CI-MPR to lysosomes, probably by sequestration into endosome-derived tubules from where the receptor returns to the TGN... - Gga2 mediates sequential ubiquitin-independent and ubiquitin-dependent steps in the trafficking of ARN1 from the trans-Golgi network to the vacuoleYi Deng
Liver Diseases Branch, NIDDK, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 284:23830-41. 2009..These studies suggest that Gga2 is involved in sorting at both the TGN and multivesicular body and that the first step can occur without ubiquitin binding... - BLOC-3, a protein complex containing the Hermansky-Pudlak syndrome gene products HPS1 and HPS4Jose A Martina
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 278:29376-84. 2003.... - Ubiquitin binding and conjugation regulate the recruitment of Rabex-5 to early endosomesRafael Mattera
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
EMBO J 27:2484-94. 2008..We also show that monoubiquitinated Rabex-5 is enriched in the cytosol. These observations are consistent with a model whereby a cycle of Ub binding and monoubiquitination regulates the association of Rabex-5 with endosomes... - Mechanisms of CD4 downregulation by the Nef and Vpu proteins of primate immunodeficiency virusesO Wolf Lindwasser
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Curr Mol Med 7:171-84. 2007..These two mechanisms lead to a dramatic reduction of CD4 expression in infected cells and are essential for efficient virus replication and disease progression... - Role of the endocytic machinery in the sorting of lysosome-associated membrane proteinsKaty Janvier
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 16:4231-42. 2005..Because AP-2 is exclusively associated with plasma membrane clathrin coats, our observations imply that a significant population of Lamps traffic via the plasma membrane en route to lysosomes... - Transmembrane domain determinants of CD4 Downregulation by HIV-1 VpuJavier G Magadán
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
J Virol 86:757-72. 2012..These findings highlight the exploitation of several TMD-mediated mechanisms by HIV-1 Vpu in order to downregulate CD4 and thus promote viral pathogenesis... - Epidermal growth factor-dependent phosphorylation of the GGA3 adaptor protein regulates its recruitment to membranesSatoshi Kametaka
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T Room 101, National Institutes of Health, Bethesda, MD 20892, USA
Mol Cell Biol 25:7988-8000. 2005..These observations indicate that EGF signaling elicits phosphorylation events that regulate the association of GGA3 with organellar membranes... - Divalent interaction of the GGAs with the Rabaptin-5-Rabex-5 complexRafael Mattera
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
EMBO J 22:78-88. 2003..These observations thus identify a binding sequence for GAE/gamma-adaptin ear domains and reveal a functional link between proteins regulating TGN cargo export and endosomal tethering/fusion events... - Conservation and diversification of dileucine signal recognition by adaptor protein (AP) complex variantsRafael Mattera
Cell Biology and Metabolism Program, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 286:2022-30. 2011..However, the characteristics of the binding site on each complex vary, providing for the specific recognition of a diverse repertoire of (D/E)XXXL(L/I) signals... - Lysosome-related organellesE C Dell'Angelica
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 5430, USA
FASEB J 14:1265-78. 2000.... - The retromer subunit Vps26 has an arrestin fold and binds Vps35 through its C-terminal domainHang Shi
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, US National Institutes of Health, US Department of Health and Human Services, Bethesda, Maryland 20892, USA
Nat Struct Mol Biol 13:540-8. 2006..Hydrophobic residues and a glycine in this loop are required for integration into the retromer complex and endosomal localization of human Vps26, and for the function of yeast Vps26 in carboxypeptidase Y sorting... - In vitro assays of Arf1 interaction with GGA proteinsHye-Young Yoon
Laboratory of Cellular Oncology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
Methods Enzymol 404:316-32. 2005..In this chapter, we describe in vitro assays for analysis of GGA interaction with Arf1 x GTP and for determining intracellular Arf1 x GTP levels... - Serine residues in the cytosolic tail of the T-cell antigen receptor alpha-chain mediate ubiquitination and endoplasmic reticulum-associated degradation of the unassembled proteinShuhei Ishikura
Cell Biology and Metabolism Program, Eunice Kennedy Shriver NICHD, Bethesda, MD 20892, USA
J Biol Chem 285:23916-24. 2010..We also found that this ubiquitination was mediated by the ER-localized ubiquitin ligase, HRD1. These findings indicate that serine-dependent, HRD1-mediated ubiquitination targets TCRalpha to the ERAD pathway... - Adaptins: the final recountM Boehm
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Biol Cell 12:2907-20. 2001..Finally, we extend our survey to adaptin-related proteins such as the GGAs and stonins, which contain domains homologous to the adaptins... 
Structural basis for recruitment and activation of the AP-1 clathrin adaptor complex by Arf1Xuefeng Ren
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Cell 152:755-67. 2013..A third Arf1 interaction site near the N terminus of the γ subunit is important for recruitment, but not activation. These observations lead to a model for the recruitment and activation of AP-1 by Arf1...- Crystallographic and functional analysis of the ESCRT-I /HIV-1 Gag PTAP interactionYoung Jun Im
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892 0580, USA
Structure 18:1536-47. 2010..However, the mutant alleles did rescue downregulation of endogenous EGF receptor. This demonstrates that the PSAP motif is not rate determining in EGF receptor downregulation under normal conditions... - Structural basis for acidic-cluster-dileucine sorting-signal recognition by VHS domainsSaurav Misra
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
Nature 415:933-7. 2002..The rigid spatial alignment of the three binding subsites leads to high specificity... - Coat proteins: shaping membrane transportJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T/Room 101, National Institutes of Health, Bethesda, Maryland 20892, USA
Nat Rev Mol Cell Biol 4:409-14. 2003.... - Specific regulation of the adaptor protein complex AP-3 by the Arf GAP AGAP1Zhongzhen Nie
Laboratory of Cellular Oncology, Center for Cancer Research, National Cancer Institute, Building 37, Room 4118, Bethesda, MD 20892, USA
Dev Cell 5:513-21. 2003..Cells overexpressing AGAP1 also exhibit increased LAMP1 trafficking via the plasma membrane. Taken together, these results support the hypothesis that AGAP1 directly and specifically regulates AP-3-dependent trafficking... - Structural requirements for function of yeast GGAs in vacuolar protein sorting, alpha-factor maturation, and interactions with clathrinC Mullins
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 5430, USA
Mol Cell Biol 21:7981-94. 2001..Thus, these observations demonstrate that the yeast GGAs play important roles in the CPY pathway, vacuole biogenesis, and alpha-factor maturation and identify structural determinants that are critical for these functions... - Signal-mediated, AP-1/clathrin-dependent sorting of transmembrane receptors to the somatodendritic domain of hippocampal neuronsGinny G Farías
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Neuron 75:810-23. 2012..These findings highlight the primary recognition event that underlies somatodendritic sorting and contribute to the evolving view of AP-1 as a global regulator of cell polarity... - Dual roles of the mammalian GARP complex in tethering and SNARE complex assembly at the trans-golgi networkF Javier Pérez-Victoria
Cell Biology and Metabolism Program, NICHD, Building 18T Room 101, NIH, Bethesda, MD 20892, USA
Mol Cell Biol 29:5251-63. 2009..These findings indicate that GARP orchestrates retrograde transport from endosomes to the TGN by promoting vesicle tethering and assembly of SNARE complexes in consecutive, independent steps... - A tubular EHD1-containing compartment involved in the recycling of major histocompatibility complex class I molecules to the plasma membraneSteve Caplan
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
EMBO J 21:2557-67. 2002..These observations suggest an additional function of EHD1 as a tubule-inducing factor in the Arf6 pathway for recycling of plasma membrane proteins internalized by clathrin-independent endocytosis... - The contribution of VHL substrate binding and HIF1-alpha to the phenotype of VHL loss in renal cell carcinomaJodi K Maranchie
Urologic Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA
Cancer Cell 1:247-55. 2002..We further demonstrate that normoxic stabilization of HIF1alpha alone, while capable of mimicking some aspects of VHL loss, is not sufficient to reproduce tumorigenesis, indicating that it is not the critical oncogenic substrate of VHL... - Pallidin is a component of a multi-protein complex involved in the biogenesis of lysosome-related organellesKengo Moriyama
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Traffic 3:666-77. 2002..These observations indicate that pallidin is a subunit of a novel multi-protein complex involved in the biogenesis of lysosome-related organelles... - Distinct requirements for the AP-3 adaptor complex in pigment granule and synaptic vesicle biogenesis in Drosophila melanogasterC Mullins
Cell Biology and Metabolism Branch, National Institutes of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Gen Genet 263:1003-14. 2000..Together, these results demonstrate that Drosophila AP-3 is critical for the biogenesis of pigment granules, but is apparently not essential for formation of a major population of synaptic vesicles in vivo... - Defective expression of the mu3 subunit of the AP-3 adaptor complex in the Drosophila pigmentation mutant carmineC Mullins
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Mol Gen Genet 262:401-12. 1999..These findings provide evidence of a role for mu3 in the sorting processes required for pigment granule biogenesis in Drosophila... - Molecular characterization of hepatocystin, the protein that is defective in autosomal dominant polycystic liver diseaseJoost P H Drenth
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 18T, Room 101, Bethesda, MD 20892, USA
Gastroenterology 126:1819-27. 2004..Thus, the exact localization and cellular function of hepatocystin remain unclear... - Genetic analyses of adaptin function from yeast to mammalsMarkus Boehm
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Building 18T Room 101, National Institutes of Health, Bethesda, MD 20892, USA
Gene 286:175-86. 2002..Here, we review recent progress in the genetic analysis of the function of AP complexes, focusing on studies that make use of targeted interference or naturally-occurring mutations in various model organisms... - Human Vam6p promotes lysosome clustering and fusion in vivoS Caplan
Cell Biology and Metabolism Branch at the National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Cell Biol 154:109-22. 2001..This study implicates hVam6p as a mammalian tethering/docking factor characterized with intrinsic ability to promote lysosome fusion in vivo... - Sorting of mannose 6-phosphate receptors mediated by the GGAsR Puertollano
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Science 292:1712-6. 2001..A dominant-negative GGA mutant blocked exit of the receptors from the trans-Golgi network. Thus, the GGAs appear to mediate sorting of the mannose 6-phosphate receptors at the trans-Golgi network... - The molecular machinery for lysosome biogenesisC Mullins
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Bioessays 23:333-43. 2001..Other recently identified components, however, appear to be unique to higher eukaryotes. BioEssays 23:333-343, 2001. Published 2001 John Wiley & Sons, Inc... - Altered trafficking of lysosomal proteins in Hermansky-Pudlak syndrome due to mutations in the beta 3A subunit of the AP-3 adaptorE C Dell'Angelica
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Mol Cell 3:11-21. 1999..Our results suggest that AP-3 functions in protein sorting to lysosomes and provide an example of a human disease in which altered trafficking of integral membrane proteins is due to mutations in a component of the sorting machinery... - Functional and physical interactions of the adaptor protein complex AP-4 with ADP-ribosylation factors (ARFs)M Boehm
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
EMBO J 20:6265-76. 2001..These observations establish AP-4 as an ARF1 effector and suggest a novel mode of interaction between ARF1 and an AP complex involving both constitutive and regulated interactions... - Interaction of tyrosine-based sorting signals with clathrin-associated proteinsH Ohno
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Science 269:1872-5. 1995..The interaction was confirmed by in vitro binding assays. Thus, it is likely that the medium chains serve as signal-binding components of the clathrin-dependent sorting machinery... - The GGAs promote ARF-dependent recruitment of clathrin to the TGNR Puertollano
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, Bethesda, MD 20892, USA
Cell 105:93-102. 2001..These observations suggest that the GGAs could function to link clathrin to membrane-bound ARF.GTP... - Altered expression of a novel adaptin leads to defective pigment granule biogenesis in the Drosophila eye color mutant garnetC E Ooi
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
EMBO J 16:4508-18. 1997..Thus, the eye pigmentation defect in the Drosophila garnet mutant may be attributed to compromised function of a coat protein involved in intracellular transport processes required for biogenesis or function of pigment granules... - AP-4, a novel protein complex related to clathrin adaptorsE C Dell'Angelica
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 274:7278-85. 1999..We propose that, like the related AP-1, AP-2, and AP-3 complexes, AP-4 plays a role in signal-mediated trafficking of integral membrane proteins in mammalian cells... - Cloning of the gene encoding the murine clathrin-associated adaptor medium chain mu 2: gene organization, alternative splicing and chromosomal assignmentH Ohno
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Gene 210:187-93. 1998..The isolation and characterization of the mu 2 gene should be instrumental for future studies of the genetics and physiological role of the adaptor medium chains in mammals... - Signal-binding specificity of the mu4 subunit of the adaptor protein complex AP-4R C Aguilar
Cell Biology and Metabolism Branch and the Laboratory of Molecular Genetics, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 276:13145-52. 2001..This chimera was targeted to the endosomal-lysosomal system without being internalized from the plasma membrane... - Endosome-specific localization and function of the ARF activator GNOMJuan S Bonifacino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Cell 112:141-2. 2003..In this issue of Cell, Geldner et al. demonstrate that the Arabidopsis ARF activator GNOM localizes to endosomes where it controls the polarized trafficking of the auxin efflux carrier PIN1 to the basal plasma membrane... - Genomic screen for vacuolar protein sorting genes in Saccharomyces cerevisiaeCecilia J Bonangelino
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Mol Biol Cell 13:2486-501. 2002..The identification and phenotypic characterization of these novel mutants provide new insights into the mechanisms of vacuolar protein sorting, most notably the probable involvement of the actin cytoskeleton in this process... - Association of the AP-3 adaptor complex with clathrinE C Dell'Angelica
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Science 280:431-4. 1998..AP-3 colocalized with clathrin in cells as observed by immunofluorescence and immunoelectron microscopy. Thus, AP-3 function in protein sorting may depend on clathrin... - T cell CD3-zeta eta heterodimer expression and coupling to phosphoinositide hydrolysisM Mercep
Biological Response Modifiers Program, National Cancer Institute, Bethesda, MD 20892
Science 242:571-4. 1988.... - ADP-Ribosylation factor 1 (ARF1) regulates recruitment of the AP-3 adaptor complex to membranesC E Ooi
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health NIH, Bethesda, Maryland 20892, USA
J Cell Biol 142:391-402. 1998..This finding suggests that ARF1 is not a regulator of specific coat proteins, but rather is a ubiquitous molecular switch that acts as a transducer of diverse signals influencing coat assembly... - ImmunoprecipitationJ S Bonifacino
National Institute of Child Health and Human Development, Bethesda, Maryland, USA
Curr Protoc Cell Biol . 2001..An additional method is provided for increasing the specificity of the technique by reprecipitating the antigen with the same or a different antibody... - Molecular characterization of the protein encoded by the Hermansky-Pudlak syndrome type 1 geneE C Dell'Angelica
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 275:1300-6. 2000.... - Regulating the retention of T-cell receptor alpha chain variants within the endoplasmic reticulum: Ca(2+)-dependent association with BiPC K Suzuki
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892
J Cell Biol 114:189-205. 1991..These data suggest that the intracellular fate of newly synthesized proteins stably associated with BiP can be regulated by Ca2+ levels in the ER... - AP-3: an adaptor-like protein complex with ubiquitous expressionE C Dell'Angelica
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
EMBO J 16:917-28. 1997..These results suggest that the sigma3 chains are components of a novel, ubiquitous adaptor-like complex involved in the recognition of tyrosine-based sorting signals... - Beta3A-adaptin, a subunit of the adaptor-like complex AP-3E C Dell'Angelica
Cell Biology and Metabolism Branch, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 272:15078-84. 1997..The characteristics of beta3A-adaptin reported here lend support to the idea that AP-3 is a structural and functional homolog of the clathrin-associated adaptors AP-1 and AP-2... - Interaction of endocytic signals from the HIV-1 envelope glycoprotein complex with members of the adaptor medium chain familyH Ohno
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Virology 238:305-15. 1997..These observations suggest that HIV-1 Env utilizes the protein sorting machinery of the host cells for internalization and sorting at various steps of the endocytic and biosynthetic pathways... - Localization of endogenous furin in cultured cell linesJ Shapiro
Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
J Histochem Cytochem 45:3-12. 1997..Therefore, our results suggest that endogenous furin is predominantly localized to the area of the Golgi complex, most likely within the trans-Golgi network... - Metabolic labeling with amino acidsJ S Bonifacino
National Institute of Child Health and Human Development, Bethesda, Maryland, USA
Curr Protoc Cell Biol . 2001..Three procedures are described for suspension and adherent cells: pulse-labeling, pulse-chase labeling, or continuous long-term labeling. A support protocol describes TCA precipitation to measure the extent of labeling... - ImmunoprecipitationJ S Bonifacino
National Institute of Child Health and Human Development, Bethesda, Maryland, USA
Curr Protoc Mol Biol . 2001..g., with and without detergent, using glass beads, etc.). Flow charts and figures give the user a clear-cut explanation of the options for employing the technology... - Metabolic labeling with amino acidsJ S Bonifacino
National Institute of Child Health and Human Development, Bethesda, Maryland, USA
Curr Protoc Mol Biol . 2001.... - ImmunoprecipitationJ S Bonifacino
National Institute of Child Health and Human Development, Bethesda, Maryland, USA
Curr Protoc Protein Sci . 2001..g., with and without detergent, using glass beads, etc.). Flow charts and figures give the user a clear-cut explanation of the options for employing the technology... - Biosynthetic labeling of proteinsJ S Bonifacino
National Institute of Child Health and Human Development, Bethesda, Maryland, USA
Curr Protoc Immunol . 2001..Label incorporation can be determined as described in a support protocol. The cells used in these protocols can be either from suspension cultures or from single-cell suspensions of spleen or thymus...