PubRank

William E Balch

Author PubWeight™ 106.10‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Biological and chemical approaches to diseases of proteostasis deficiency. Annu Rev Biochem 2009 5.23
2 Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 2006 5.01
3 Functional amyloid--from bacteria to humans. Trends Biochem Sci 2007 4.50
4 Functional amyloid formation within mammalian tissue. PLoS Biol 2006 3.92
5 Structural basis for cargo regulation of COPII coat assembly. Cell 2008 3.15
6 Chemical and biological approaches synergize to ameliorate protein-folding diseases. Cell 2008 3.11
7 The biological and chemical basis for tissue-selective amyloid disease. Cell 2005 2.76
8 Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease. Proc Natl Acad Sci U S A 2002 2.71
9 Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol 2009 2.70
10 Structure of the Sec13/31 COPII coat cage. Nature 2006 2.63
11 Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants. ACS Chem Biol 2006 2.25
12 Domain interdependence in the biosynthetic assembly of CFTR. J Mol Biol 2006 2.17
13 The COPII cage: unifying principles of vesicle coat assembly. Nat Rev Mol Cell Biol 2006 1.88
14 An adaptable standard for protein export from the endoplasmic reticulum. Cell 2007 1.86
15 COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code. J Cell Biol 2004 1.70
16 Small-molecule proteostasis regulators for protein conformational diseases. Nat Chem Biol 2011 1.64
17 Geranylgeranyl switching regulates GDI-Rab GTPase recycling. Structure 2003 1.63
18 Amylin proprotein processing generates progressively more amyloidogenic peptides that initially sample the helical state. Biochemistry 2008 1.51
19 Diversity in the origins of proteostasis networks--a driver for protein function in evolution. Nat Rev Mol Cell Biol 2013 1.51
20 Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway. J Biol Chem 2002 1.50
21 Large-scale profiling of Rab GTPase trafficking networks: the membrome. Mol Biol Cell 2005 1.49
22 Structural design of cage and coat scaffolds that direct membrane traffic. Curr Opin Struct Biol 2007 1.44
23 Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis. Mol Biol Cell 2010 1.41
24 Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic 2008 1.34
25 The delta subunit of AP-3 is required for efficient transport of VSV-G from the trans-Golgi network to the cell surface. Proc Natl Acad Sci U S A 2002 1.32
26 The proteostasis boundary in misfolding diseases of membrane traffic. FEBS Lett 2009 1.31
27 Heparin accelerates gelsolin amyloidogenesis. Biochemistry 2006 1.31
28 Rab-alphaGDI activity is regulated by a Hsp90 chaperone complex. EMBO J 2002 1.26
29 Amyloid as a natural product. J Cell Biol 2003 1.25
30 A new pharmacology--drugging stressed folding pathways. Trends Mol Med 2005 1.23
31 Modeling general proteostasis: proteome balance in health and disease. Curr Opin Cell Biol 2010 1.13
32 Discovery and characterization of a mammalian amyloid disaggregation activity. Protein Sci 2010 1.13
33 The Hsp90 chaperone complex regulates GDI-dependent Rab recycling. Mol Biol Cell 2006 1.09
34 Pathological and functional amyloid formation orchestrated by the secretory pathway. Curr Opin Struct Biol 2003 1.02
35 Di-acidic motifs in the membrane-distal C termini modulate the transport of angiotensin II receptors from the endoplasmic reticulum to the cell surface. J Biol Chem 2011 1.02
36 Gelsolin amyloidosis: genetics, biochemistry, pathology and possible strategies for therapeutic intervention. Crit Rev Biochem Mol Biol 2012 1.01
37 Metalloendoprotease cleavage triggers gelsolin amyloidogenesis. EMBO J 2005 1.00
38 NPC1/NPC2 function as a tag team duo to mobilize cholesterol. Proc Natl Acad Sci U S A 2008 1.00
39 Histone deacetylase inhibitor (HDACi) suberoylanilide hydroxamic acid (SAHA)-mediated correction of α1-antitrypsin deficiency. J Biol Chem 2012 0.98
40 Secretion of amyloidogenic gelsolin progressively compromises protein homeostasis leading to the intracellular aggregation of proteins. Proc Natl Acad Sci U S A 2009 0.96
41 Cell Biology. The proteome in balance. Science 2010 0.95
42 Blue journal conference. Aging and susceptibility to lung disease. Am J Respir Crit Care Med 2015 0.94
43 Structural and functional analysis of the globular head domain of p115 provides insight into membrane tethering. J Mol Biol 2009 0.94
44 The role of ARF1 and rab GTPases in polarization of the Golgi stack. Traffic 2005 0.94
45 A chaperone trap contributes to the onset of cystic fibrosis. PLoS One 2012 0.93
46 The integration of cell and chemical biology in protein folding. Nat Chem Biol 2006 0.93
47 Molecular evolution of the Rab-escort-protein/guanine-nucleotide-dissociation-inhibitor superfamily. Mol Biol Cell 2003 0.92
48 An evolutionary perspective on eukaryotic membrane trafficking. Adv Exp Med Biol 2007 0.92
49 The lysophospholipid acyltransferase antagonist CI-976 inhibits a late step in COPII vesicle budding. Traffic 2008 0.92
50 Gelsolin domain 2 Ca2+ affinity determines susceptibility to furin proteolysis and familial amyloidosis of finnish type. J Mol Biol 2003 0.91
51 Costly mistakes: translational infidelity and protein homeostasis. Cell 2008 0.91
52 Compromised mutant EFEMP1 secretion associated with macular dystrophy remedied by proteostasis network alteration. Mol Biol Cell 2011 0.90
53 Expanding proteostasis by membrane trafficking networks. Cold Spring Harb Perspect Biol 2013 0.89
54 Translational attenuation differentially alters the fate of disease-associated fibulin proteins. FASEB J 2012 0.88
55 Emergent properties of proteostasis-COPII coupled systems in human health and disease. Mol Membr Biol 2010 0.88
56 Protein energetics in maturation of the early secretory pathway. Curr Opin Cell Biol 2007 0.87
57 Proteostasis: a new therapeutic paradigm for pulmonary disease. Proc Am Thorac Soc 2011 0.86
58 The 8 and 5 kDa fragments of plasma gelsolin form amyloid fibrils by a nucleated polymerization mechanism, while the 68 kDa fragment is not amyloidogenic. Biochemistry 2009 0.85
59 Proteostasis strategies for restoring alpha1-antitrypsin deficiency. Proc Am Thorac Soc 2010 0.84
60 Potential Agents for Treating Cystic Fibrosis: Cyclic Tetrapeptides that Restore Trafficking and Activity of ΔF508-CFTR. ACS Med Chem Lett 2011 0.82
61 FK506 binding protein 8 peptidylprolyl isomerase activity manages a late stage of cystic fibrosis transmembrane conductance regulator (CFTR) folding and stability. J Biol Chem 2012 0.82
62 A general strategy for the bacterial expression of amyloidogenic peptides using BCL-XL-1/2 fusions. Protein Sci 2009 0.81
63 Mise en place-this bud's for the Golgi. Mol Cell 2004 0.81
64 Insulin biosynthetic interaction network component, TMEM24, facilitates insulin reserve pool release. Cell Rep 2013 0.80
65 Diffuse lung disease in children: summary of a scientific conference. Pediatr Pulmonol 2013 0.79
66 Rab1b silencing using small interfering RNA for analysis of disease-specific function. Methods Enzymol 2008 0.79
67 CFTR Folding Consortium: methods available for studies of CFTR folding and correction. Methods Mol Biol 2011 0.79
68 Q-bodies monitor the quinary state of the protein fold. Nat Cell Biol 2013 0.78
69 Use of Hsp90 inhibitors to disrupt GDI-dependent Rab recycling. Methods Enzymol 2005 0.77
70 Ca2+ binding protects against gelsolin amyloidosis. Biochem Biophys Res Commun 2004 0.77
71 Exploring trafficking GTPase function by mRNA expression profiling: use of the SymAtlas web-application and the Membrome datasets. Methods Enzymol 2005 0.77
72 Protein folding: Protection from the outside. Nature 2011 0.77
73 Recombinant production in baculovirus-infected insect cells and purification of the mammalian Sec13/Sec31 complex. Methods Enzymol 2005 0.76
74 Quantitative proteomic profiling reveals differentially regulated proteins in cystic fibrosis cells. J Proteome Res 2014 0.76
75 Protein folding: salty sea regulators of cystic fibrosis. Nat Chem Biol 2013 0.75
76 Formation of gelsolin amyloid fibrils in the rough endoplasmic reticulum of skeletal muscle in the gelsolin mouse model of inclusion body myositis: comparative analysis to human sporadic inclusion body myositis. Ultrastruct Pathol 2013 0.75
77 Expanding proteostasis by membrane trafficking networks. Cold Spring Harb Perspect Med 2013 0.75
78 Purification and properties of mammalian Sec23/24 from insect cells. Methods Enzymol 2005 0.75
79 Protein engineering for crystallization of the GTPase Sar1 that regulates ER vesicle budding. Acta Crystallogr D Biol Crystallogr 2002 0.75