Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer.

PubWeight™: 2.53‹?› | Rank: Top 2%

🔗 View Article (PMC 2668050)

Published in Am J Hum Genet on December 18, 2008

Authors

Yongyu Wang1, Phillip J Kuan, Chao Xing, Jennifer T Cronkhite, Fernando Torres, Randall L Rosenblatt, J Michael DiMaio, Lisa N Kinch, Nick V Grishin, Christine Kim Garcia

Author Affiliations

1: Eugene McDermott Center for Human Growth and Development, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.

Articles citing this

A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med (2011) 6.40

A genome-wide association study of lung cancer identifies a region of chromosome 5p15 associated with risk for adenocarcinoma. Am J Hum Genet (2009) 5.62

Strategies for treating idiopathic pulmonary fibrosis. Nat Rev Drug Discov (2010) 3.20

Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet (2013) 3.17

Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA (2013) 2.20

Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease. Annu Rev Med (2010) 2.05

Pulmonary fibrosis: patterns and perpetrators. J Clin Invest (2012) 2.05

Telomerase reverse transcriptase locus polymorphisms and cancer risk: a field synopsis and meta-analysis. J Natl Cancer Inst (2012) 1.82

Genetic disorders of surfactant dysfunction. Pediatr Dev Pathol (2009) 1.77

Future directions in idiopathic pulmonary fibrosis research. An NHLBI workshop report. Am J Respir Crit Care Med (2014) 1.54

Pathogenesis of idiopathic pulmonary fibrosis. Annu Rev Pathol (2013) 1.51

Exome sequencing links mutations in PARN and RTEL1 with familial pulmonary fibrosis and telomere shortening. Nat Genet (2015) 1.46

Subclinical lung disease, macrocytosis, and premature graying in kindreds with telomerase (TERT) mutations. Chest (2011) 1.42

Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress. J Biol Chem (2010) 1.38

Pulmonary surfactant: an immunological perspective. Cell Physiol Biochem (2009) 1.34

Animal models of fibrotic lung disease. Am J Respir Cell Mol Biol (2013) 1.31

Emerging evidence for endoplasmic reticulum stress in the pathogenesis of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol (2012) 1.31

Genetic Basis of Children's Interstitial Lung Disease. Pediatr Allergy Immunol Pulmonol (2010) 1.23

Idiopathic pulmonary fibrosis: update on genetic discoveries. Proc Am Thorac Soc (2011) 1.23

Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis. Dis Model Mech (2013) 1.21

Epidemiology of idiopathic pulmonary fibrosis. Clin Epidemiol (2013) 1.17

Surfactant dysfunction. Paediatr Respir Rev (2011) 1.16

Rare variants in RTEL1 are associated with familial interstitial pneumonia. Am J Respir Crit Care Med (2015) 1.15

TGFβ signaling in lung epithelium regulates bleomycin-induced alveolar injury and fibroblast recruitment. Am J Physiol Lung Cell Mol Physiol (2011) 1.14

EMT and interstitial lung disease: a mysterious relationship. Curr Opin Pulm Med (2012) 1.12

Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction. Am J Med Sci (2011) 1.10

Biomarkers in idiopathic pulmonary fibrosis. Curr Opin Pulm Med (2012) 1.09

Implicating exudate macrophages and Ly-6C(high) monocytes in CCR2-dependent lung fibrosis following gene-targeted alveolar injury. J Immunol (2013) 1.09

Extensive phenotyping of individuals at risk for familial interstitial pneumonia reveals clues to the pathogenesis of interstitial lung disease. Am J Respir Crit Care Med (2015) 1.04

Sequencing of idiopathic pulmonary fibrosis-related genes reveals independent single gene associations. BMJ Open Respir Res (2014) 1.04

Interstitial lung disease: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases. Ann Am Thorac Soc (2014) 1.04

Endoplasmic reticulum stress as a pro-fibrotic stimulus. Biochim Biophys Acta (2012) 1.03

New therapeutic targets in idiopathic pulmonary fibrosis. Aiming to rein in runaway wound-healing responses. Am J Respir Crit Care Med (2014) 1.02

A novel dyskerin (DKC1) mutation is associated with familial interstitial pneumonia. Chest (2014) 1.01

Allele-specific transactivation of matrix metalloproteinase 7 by FOXA2 and correlation with plasma levels in idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol (2012) 1.01

Diseases of pulmonary surfactant homeostasis. Annu Rev Pathol (2015) 1.01

Molecular biomarkers in idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol (2014) 0.99

Genetics in pulmonary fibrosis--familial cases provide clues to the pathogenesis of idiopathic pulmonary fibrosis. Am J Med Sci (2011) 0.98

Exome sequencing identifies mutant TINF2 in a family with pulmonary fibrosis. Chest (2015) 0.97

Large-scale RNA-Seq Transcriptome Analysis of 4043 Cancers and 548 Normal Tissue Controls across 12 TCGA Cancer Types. Sci Rep (2015) 0.95

A large kindred of pulmonary fibrosis associated with a novel ABCA3 gene variant. Respir Res (2014) 0.94

Pathogenesis of Interstitial Lung Disease in Children and Adults. Pediatr Allergy Immunol Pulmonol (2010) 0.93

Association between MUC5B and TERT polymorphisms and different interstitial lung disease phenotypes. Transl Res (2013) 0.93

Lung cancer and interstitial lung diseases: a systematic review. Pulm Med (2012) 0.92

Ethnic and racial differences in the presence of idiopathic pulmonary fibrosis at death. Respir Med (2012) 0.91

Ecogenomics of respiratory diseases of public health significance. Annu Rev Public Health (2010) 0.91

The genetic basis of idiopathic pulmonary fibrosis. Eur Respir J (2015) 0.91

Retracted Mutant surfactant A2 proteins associated with familial pulmonary fibrosis and lung cancer induce TGF-β1 secretion. Proc Natl Acad Sci U S A (2012) 0.89

Genetic Evaluation and Testing of Patients and Families with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med (2016) 0.89

Genetic interstitial lung disease. Clin Chest Med (2011) 0.89

A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features. Respir Res (2012) 0.88

Epidermal growth factor receptor (EGFR) pathway genes and interstitial lung disease: an association study. Sci Rep (2014) 0.87

The UPR and lung disease. Semin Immunopathol (2013) 0.86

CT scan findings of probable usual interstitial pneumonitis have a high predictive value for histologic usual interstitial pneumonitis. Chest (2015) 0.86

A novel telomerase activator suppresses lung damage in a murine model of idiopathic pulmonary fibrosis. PLoS One (2013) 0.85

Personalized medicine: applying 'omics' to lung fibrosis. Biomark Med (2012) 0.84

Idiopathic Pulmonary Fibrosis: A Genetic Disease That Involves Mucociliary Dysfunction of the Peripheral Airways. Physiol Rev (2016) 0.84

Lost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease. Am J Physiol Lung Cell Mol Physiol (2015) 0.83

Epigenetics of idiopathic pulmonary fibrosis. Transl Res (2014) 0.83

Single-cell RNA sequencing identifies diverse roles of epithelial cells in idiopathic pulmonary fibrosis. JCI Insight (2016) 0.82

TERT genetic polymorphism rs2736100 was associated with lung cancer: a meta-analysis based on 14,492 subjects. Genet Test Mol Biomarkers (2013) 0.82

Incorporating genetics into the identification and treatment of Idiopathic Pulmonary Fibrosis. BMC Med (2015) 0.82

Familial interstitial pulmonary fibrosis: a large family with atypical clinical features. Can Respir J (2010) 0.82

The Role of Surfactant in Lung Disease and Host Defense against Pulmonary Infections. Ann Am Thorac Soc (2015) 0.81

Use of a genealogical database demonstrates heritability of pulmonary fibrosis. Lung (2013) 0.81

Genetics of Interstitial Lung Disease: Vol de Nuit (Night Flight). Clin Med Insights Circ Respir Pulm Med (2015) 0.81

Germline mutations predisposing to non-small cell lung cancer. Fam Cancer (2015) 0.80

Telomeres revisited: RTEL1 variants in pulmonary fibrosis. Eur Respir J (2015) 0.79

Germline mutations causing familial lung cancer. J Hum Genet (2015) 0.79

An Exome Sequencing Study to Assess the Role of Rare Genetic Variation in Pulmonary Fibrosis. Am J Respir Crit Care Med (2017) 0.79

Genome sequencing of idiopathic pulmonary fibrosis in conjunction with a medical school human anatomy course. PLoS One (2014) 0.79

Basal cells of the human airways acquire mesenchymal traits in idiopathic pulmonary fibrosis and in culture. Lab Invest (2015) 0.79

Sertoli cell-mediated differentiation of male germ cell-like cells from human umbilical cord Wharton's jelly-derived mesenchymal stem cells in an in vitro co-culture system. Eur J Med Res (2015) 0.79

Diffuse lung disease in children: summary of a scientific conference. Pediatr Pulmonol (2013) 0.79

Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy. J Biol Chem (2015) 0.79

Retracted Lung fibrosis-associated surfactant protein A1 and C variants induce latent transforming growth factor β1 secretion in lung epithelial cells. J Biol Chem (2013) 0.78

Differential telomerase expression in idiopathic pulmonary fibrosis and non-small cell lung cancer. Oncol Rep (2013) 0.78

MUC5B and Idiopathic Pulmonary Fibrosis. Ann Am Thorac Soc (2015) 0.78

Human surfactant protein A2 gene mutations impair dimmer/trimer assembly leading to deficiency in protein sialylation and secretion. PLoS One (2012) 0.78

DNA methylation profile and expression of surfactant protein A2 gene in lung cancer. Exp Lung Res (2014) 0.77

Taking the "I" out of IPF. Eur Respir J (2015) 0.77

Bronchoalveolar lavage and other methods to define the human respiratory tract milieu in health and disease. Lung (2011) 0.77

Genetic architecture of human fibrotic diseases: disease risk and disease progression. Front Pharmacol (2013) 0.76

Candidate genes of idiopathic pulmonary fibrosis: current evidence and research. Appl Clin Genet (2016) 0.76

Genome-wide imputation study identifies novel HLA locus for pulmonary fibrosis and potential role for auto-immunity in fibrotic idiopathic interstitial pneumonia. BMC Genet (2016) 0.76

Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis. J Clin Invest (2017) 0.75

Chlorambucil-induced acute interstitial pneumonitis. Case Rep Hematol (2014) 0.75

Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations. Clin Med Insights Circ Respir Pulm Med (2016) 0.75

CT Imaging Phenotypes of Pulmonary Fibrosis in the MUC5B Promoter Site Polymorphism. Chest (2016) 0.75

Pharmacogenetics and interstitial lung disease. Curr Opin Pulm Med (2016) 0.75

Candidate gene analysis of the surfactant protein D gene in pediatric diffuse lung disease. J Pediatr (2013) 0.75

IDIOPATHIC PULMONARY FIBROSIS IS A COMPLEX GENETIC DISORDER. Trans Am Clin Climatol Assoc (2016) 0.75

Familial Interstitial Pneumonia (FIP). Clin Pulm Med (2014) 0.75

Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs. Biomed Res Int (2015) 0.75

EZH2 enhances the differentiation of fibroblasts into myofibroblasts in idiopathic pulmonary fibrosis. Physiol Rep (2016) 0.75

Genetic testing in diffuse parenchymal lung disease. Orphanet J Rare Dis (2012) 0.75

Genetics in Idiopathic Pulmonary Fibrosis Pathogenesis, Prognosis, and Treatment. Front Med (Lausanne) (2017) 0.75

Articles cited by this

Merlin--rapid analysis of dense genetic maps using sparse gene flow trees. Nat Genet (2001) 53.16

American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med (2002) 19.02

Allele-sharing models: LOD scores and accurate linkage tests. Am J Hum Genet (1997) 13.93

Low LDL cholesterol in individuals of African descent resulting from frequent nonsense mutations in PCSK9. Nat Genet (2005) 12.12

The Dallas Heart Study: a population-based probability sample for the multidisciplinary study of ethnic differences in cardiovascular health. Am J Cardiol (2004) 9.43

Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med (2007) 8.23

Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci U S A (2007) 5.95

Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med (2008) 5.82

Two distinct classes of carbohydrate-recognition domains in animal lectins. J Biol Chem (1988) 4.79

A mutation in the surfactant protein C gene associated with familial interstitial lung disease. N Engl J Med (2001) 4.68

Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred. Am J Respir Crit Care Med (2002) 4.66

Immunoregulatory functions of surfactant proteins. Nat Rev Immunol (2005) 4.55

Lung cancer and cryptogenic fibrosing alveolitis. A population-based cohort study. Am J Respir Crit Care Med (2000) 3.36

Transformation of alveolar type 2 cells to type 1 cells following exposure to NO2. Exp Mol Pathol (1975) 3.07

Molecular basis of opsonic defect in immunodeficient children. Lancet (1991) 3.00

Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis. Am J Respir Crit Care Med (2008) 2.90

Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax (2000) 2.16

Lung cancer preneoplasia. Annu Rev Pathol (2006) 2.04

A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation. Am J Respir Cell Mol Biol (2005) 1.90

Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. J Biol Chem (2003) 1.62

Surfactant protein A and surfactant protein D in health and disease. Am J Physiol (1998) 1.56

Surfactant-associated protein A inhibits LPS-induced cytokine and nitric oxide production in vivo. Am J Physiol Lung Cell Mol Physiol (2000) 1.45

Crystal structure of trimeric carbohydrate recognition and neck domains of surfactant protein A. J Biol Chem (2003) 1.37

Alveolar cell carcinoma occurring in idiopathic interstitial pulmonary fibrosis. Br J Dis Chest (1970) 1.32

Collectins: sentinels of innate immunity. Bioessays (2007) 1.20

Telomerase in alveolar epithelial development and repair. Am J Physiol Lung Cell Mol Physiol (2000) 1.15

Surfactant protein A (SP-A) gene targeted mice. Biochim Biophys Acta (1998) 1.09

Peripheral airway cell differentiation in human lung cancer cell lines. Cancer Res (1990) 1.07

Human SP-A1 and SP-A2 genes are differentially regulated during development and by cAMP and glucocorticoids. Am J Physiol (1994) 1.03

Glycosylation and secretion of surfactant-associated glycoprotein A. J Biol Chem (1985) 0.91

Simultaneous occurrence of pulmonary interstitial fibrosis and alveolar cell carcinoma in one family. Thorax (1981) 0.82

Articles by these authors

Low LDL cholesterol in individuals of African descent resulting from frequent nonsense mutations in PCSK9. Nat Genet (2005) 12.12

Substrate and functional diversity of lysine acetylation revealed by a proteomics survey. Mol Cell (2006) 11.38

A putative RNA-interference-based immune system in prokaryotes: computational analysis of the predicted enzymatic machinery, functional analogies with eukaryotic RNAi, and hypothetical mechanisms of action. Biol Direct (2006) 11.31

Genetic variation in PNPLA3 confers susceptibility to nonalcoholic fatty liver disease. Nat Genet (2008) 10.87

Dysregulation of microRNAs after myocardial infarction reveals a role of miR-29 in cardiac fibrosis. Proc Natl Acad Sci U S A (2008) 9.86

PROMALS3D: a tool for multiple protein sequence and structure alignments. Nucleic Acids Res (2008) 7.16

Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels. Cell (2012) 6.83

Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci U S A (2007) 5.95

Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med (2008) 5.82

Identification of the acyltransferase that octanoylates ghrelin, an appetite-stimulating peptide hormone. Cell (2008) 5.10

A DNA repair system specific for thermophilic Archaea and bacteria predicted by genomic context analysis. Nucleic Acids Res (2002) 4.39

Lysine acetylation is a highly abundant and evolutionarily conserved modification in Escherichia coli. Mol Cell Proteomics (2008) 4.36

Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation (2008) 4.12

Structural classification of zinc fingers: survey and summary. Nucleic Acids Res (2003) 3.94

Genome trees and the tree of life. Trends Genet (2002) 3.79

Identification of a candidate therapeutic autophagy-inducing peptide. Nature (2013) 3.76

Structure prediction for CASP8 with all-atom refinement using Rosetta. Proteins (2009) 3.64

Biochemical identification of Argonaute 2 as the sole protein required for RNA-induced silencing complex activity. Proc Natl Acad Sci U S A (2004) 3.63

AMPylation of Rho GTPases by Vibrio VopS disrupts effector binding and downstream signaling. Science (2008) 3.44

A sequence variation (I148M) in PNPLA3 associated with nonalcoholic fatty liver disease disrupts triglyceride hydrolysis. J Biol Chem (2009) 3.41

PROMALS: towards accurate multiple sequence alignments of distantly related proteins. Bioinformatics (2007) 3.26

PCMA: fast and accurate multiple sequence alignment based on profile consistency. Bioinformatics (2003) 3.23

Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet (2013) 3.17

Reprogramming of human fibroblasts toward a cardiac fate. Proc Natl Acad Sci U S A (2013) 3.14

The lipodystrophy protein seipin is found at endoplasmic reticulum lipid droplet junctions and is important for droplet morphology. Proc Natl Acad Sci U S A (2007) 3.11

Ubiquitin-induced oligomerization of the RNA sensors RIG-I and MDA5 activates antiviral innate immune response. Immunity (2012) 3.07

Transmembrane 6 superfamily member 2 gene variant disentangles nonalcoholic steatohepatitis from cardiovascular disease. Hepatology (2015) 2.81

Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med (2009) 2.65

Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulation (2013) 2.64

EGFR-mediated Beclin 1 phosphorylation in autophagy suppression, tumor progression, and tumor chemoresistance. Cell (2013) 2.63

Atypical angiopoietin-like protein that regulates ANGPTL3. Proc Natl Acad Sci U S A (2012) 2.62

Structural basis for converting a general transcription factor into an operon-specific virulence regulator. Mol Cell (2007) 2.56

Secreted kinase phosphorylates extracellular proteins that regulate biomineralization. Science (2012) 2.55

Aldehyde dehydrogenase activity selects for lung adenocarcinoma stem cells dependent on notch signaling. Cancer Res (2010) 2.54

An E3 ligase possessing an iron-responsive hemerythrin domain is a regulator of iron homeostasis. Science (2009) 2.49

Evolution of protein structures and functions. Curr Opin Struct Biol (2002) 2.38

Purified NPC1 protein: II. Localization of sterol binding to a 240-amino acid soluble luminal loop. J Biol Chem (2007) 2.34

Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. PLoS One (2010) 2.19

PSMB8 encoding the β5i proteasome subunit is mutated in joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced lipodystrophy syndrome. Am J Hum Genet (2010) 2.05

C3PO, an endoribonuclease that promotes RNAi by facilitating RISC activation. Science (2009) 2.04

Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial. Chest (2012) 2.03

The conserved plant sterility gene HAP2 functions after attachment of fusogenic membranes in Chlamydomonas and Plasmodium gametes. Genes Dev (2008) 1.95

Fido, a novel AMPylation domain common to fic, doc, and AvrB. PLoS One (2009) 1.92

CASP9 assessment of free modeling target predictions. Proteins (2011) 1.90

Thymosin beta4 mediated PKC activation is essential to initiate the embryonic coronary developmental program and epicardial progenitor cell activation in adult mice in vivo. J Mol Cell Cardiol (2009) 1.88

Detecting distant homology with Meta-BASIC. Nucleic Acids Res (2004) 1.86

MUMMALS: multiple sequence alignment improved by using hidden Markov models with local structural information. Nucleic Acids Res (2006) 1.85

Side-chain modeling with an optimized scoring function. Protein Sci (2002) 1.84

Sequence and structure classification of kinases. J Mol Biol (2002) 1.84

Admixture mapping of 15,280 African Americans identifies obesity susceptibility loci on chromosomes 5 and X. PLoS Genet (2009) 1.81

Thymosin beta4 is an essential paracrine factor of embryonic endothelial progenitor cell-mediated cardioprotection. Circulation (2008) 1.77

CASP9 target classification. Proteins (2011) 1.75

CASP5 assessment of fold recognition target predictions. Proteins (2003) 1.73

Crystal structure of human riboflavin kinase reveals a beta barrel fold and a novel active site arch. Structure (2003) 1.69

A minimal domain responsible for Munc13 activity. Nat Struct Mol Biol (2005) 1.68

Stromal cell-derived factor-1alpha is cardioprotective after myocardial infarction. Circulation (2008) 1.68

Genetic variation in ANGPTL4 provides insights into protein processing and function. J Biol Chem (2009) 1.64

Comparing on-pump and off-pump coronary artery bypass grafting: numerous studies but few conclusions: a scientific statement from the American Heart Association council on cardiovascular surgery and anesthesia in collaboration with the interdisciplinary working group on quality of care and outcomes research. Circulation (2005) 1.61

The HicAB cassette, a putative novel, RNA-targeting toxin-antitoxin system in archaea and bacteria. Bioinformatics (2006) 1.60

PROMALS3D: multiple protein sequence alignment enhanced with evolutionary and three-dimensional structural information. Methods Mol Biol (2014) 1.59

PROMALS3D web server for accurate multiple protein sequence and structure alignments. Nucleic Acids Res (2008) 1.58

NESdb: a database of NES-containing CRM1 cargoes. Mol Biol Cell (2012) 1.57

Phenotypic and genotypic analyses of genetic skin disease through the Online Mendelian Inheritance in Man (OMIM) database. J Invest Dermatol (2009) 1.57

Pharmacokinetics of oral treprostinil sustained release tablets during chronic administration to patients with pulmonary arterial hypertension. J Cardiovasc Pharmacol (2013) 1.55

Future directions in idiopathic pulmonary fibrosis research. An NHLBI workshop report. Am J Respir Crit Care Med (2014) 1.54

Kinetic and structural insights into the mechanism of AMPylation by VopS Fic domain. J Biol Chem (2010) 1.51

C/EBP transcription factors mediate epicardial activation during heart development and injury. Science (2012) 1.48

Reconstruction of ancestral protein sequences and its applications. BMC Evol Biol (2004) 1.48

Structural drift: a possible path to protein fold change. Bioinformatics (2004) 1.46

Identification of novel restriction endonuclease-like fold families among hypothetical proteins. Nucleic Acids Res (2005) 1.44

Survival in pulmonary arterial hypertension patients awaiting lung transplantation. J Heart Lung Transplant (2013) 1.43

Protein structure prediction for the male-specific region of the human Y chromosome. Proc Natl Acad Sci U S A (2004) 1.43

Prediction of functional specificity determinants from protein sequences using log-likelihood ratios. Bioinformatics (2005) 1.41

A comprehensive update of the sequence and structure classification of kinases. BMC Struct Biol (2005) 1.40

Analysis of CASP8 targets, predictions and assessment methods. Database (Oxford) (2009) 1.38

Structure of human nicotinamide/nicotinic acid mononucleotide adenylyltransferase. Basis for the dual substrate specificity and activation of the oncolytic agent tiazofurin. J Biol Chem (2002) 1.38

Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress. J Biol Chem (2010) 1.38

Nonsense mutations in folliculin presenting as isolated familial spontaneous pneumothorax in adults. Am J Respir Crit Care Med (2005) 1.37

Survey of visual and force/tactile control of robots for physical interaction in Spain. Sensors (Basel) (2009) 1.37

Realm of PD-(D/E)XK nuclease superfamily revisited: detection of novel families with modified transitive meta profile searches. BMC Struct Biol (2007) 1.33