Published in Hum Mutat on June 01, 2008
Genetic diseases of connective tissues: cellular and extracellular effects of ECM mutations. Nat Rev Genet (2009) 2.03
Unfolded protein response in fuchs endothelial corneal dystrophy: a unifying pathogenic pathway? Am J Ophthalmol (2010) 1.49
An alpha 2 collagen VIII transgenic knock-in mouse model of Fuchs endothelial corneal dystrophy shows early endothelial cell unfolded protein response and apoptosis. Hum Mol Genet (2011) 1.29
R992C (p.R1192C) Substitution in collagen II alters the structure of mutant molecules and induces the unfolded protein response. J Mol Biol (2009) 0.87
Endoplasmic reticulum stress-unfolding protein response-apoptosis cascade causes chondrodysplasia in a col2a1 p.Gly1170Ser mutated mouse model. PLoS One (2014) 0.82
The expanding spectrum of COL2A1 gene variants IN 136 patients with a skeletal dysplasia phenotype. Eur J Hum Genet (2015) 0.81
Persistence of intracellular and extracellular changes after incompletely suppressing expression of the R789C (p.R989C) and R992C (p.R1192C) collagen II mutants. Hum Mutat (2011) 0.80
ENU-induced missense mutation in the C-propeptide coding region of Col2a1 creates a mouse model of platyspondylic lethal skeletal dysplasia, Torrance type. Mamm Genome (2011) 0.79
Fluorescent protein markers to tag collagenous proteins: the paradigm of procollagen VII. Biochem Biophys Res Commun (2009) 0.77
Reducing the effects of intracellular accumulation of thermolabile collagen II mutants by increasing their thermostability in cell culture conditions. Biochem Biophys Res Commun (2010) 0.77
Endoplasmic reticulum stress in chondrodysplasias caused by mutations in collagen types II and X. Cell Stress Chaperones (2016) 0.75
Matrix-specific anchors: a new concept for targeted delivery and retention of therapeutic cells. Tissue Eng Part A (2015) 0.75
Remodeling of the dermal-epidermal junction in bilayered skin constructs after silencing the expression of the p.R2622Q and p.G2623C collagen VII mutants. Connect Tissue Res (2012) 0.75
Clinical significance of IgG deposition in the glomerular mesangial area in patients with IgA nephropathy. Clin Exp Nephrol (2012) 1.94
Candidate cell and matrix interaction domains on the collagen fibril, the predominant protein of vertebrates. J Biol Chem (2008) 1.92
Small GTPases and tyrosine kinases coregulate a molecular switch in the phosphoinositide 3-kinase regulatory subunit. Cancer Cell (2002) 1.71
Compound heterozygous desmoplakin mutations result in a phenotype with a combination of myocardial, skin, hair, and enamel abnormalities. J Invest Dermatol (2009) 1.62
Regulation of intracellular pH in human melanoma: potential therapeutic implications. Mol Cancer Ther (2002) 1.57
Direct quantification of the flexibility of type I collagen monomer. Biochem Biophys Res Commun (2002) 1.56
Inhibition of cell survival and invasive potential of colorectal carcinoma cells by the tyrosine kinase inhibitor STI571. Cancer Biol Ther (2004) 1.55
Suprabasal Dsg2 expression in transgenic mouse skin confers a hyperproliferative and apoptosis-resistant phenotype to keratinocytes. J Cell Sci (2007) 1.39
Cooperative induction of a tolerogenic dendritic cell phenotype by cytokines secreted by pancreatic carcinoma cells. J Immunol (2006) 1.33
Perlecan domain V is neuroprotective and proangiogenic following ischemic stroke in rodents. J Clin Invest (2011) 1.31
Targeting the epidermal growth factor receptor in cancer: apoptosis takes center stage. Cancer Res (2003) 1.29
Interaction with LC8 is required for Pak1 nuclear import and is indispensable for zebrafish development. PLoS One (2009) 1.29
The D2 period of collagen II contains a specific binding site for the human discoidin domain receptor, DDR2. J Mol Biol (2004) 1.23
Biochemical and structural characterization of the Pak1-LC8 interaction. J Biol Chem (2008) 1.17
Progress in epidermolysis bullosa research: toward treatment and cure. J Invest Dermatol (2010) 1.16
Selective RAF inhibitor impairs ERK1/2 phosphorylation and growth in mutant NRAS, vemurafenib-resistant melanoma cells. Pigment Cell Melanoma Res (2013) 1.13
Complex regulation of signal transducers and activators of transcription 3 activation in normal and malignant keratinocytes. Cancer Res (2004) 1.13
Novel use of zebrafish as a vertebrate model to screen radiation protectors and sensitizers. Int J Radiat Oncol Biol Phys (2005) 1.11
Resistance of Akt kinases to dephosphorylation through ATP-dependent conformational plasticity. Proc Natl Acad Sci U S A (2011) 1.09
Metastasis-associated protein (MTA)1 enhances migration, invasion, and anchorage-independent survival of immortalized human keratinocytes. Oncogene (2002) 1.08
Specific sequences in p120ctn determine subcellular distribution of its multiple isoforms involved in cellular adhesion of normal and malignant epithelial cells. J Cell Sci (2002) 1.07
Extracellular matrix protein 1 inhibits the activity of matrix metalloproteinase 9 through high-affinity protein/protein interactions. Exp Dermatol (2006) 1.07
Enhanced EGFR inhibition and distinct epitope recognition by EGFR antagonistic mAbs C225 and 425. Cancer Biol Ther (2008) 1.06
Inhibition of p73 function by Pifithrin-alpha as revealed by studies in zebrafish embryos. Cell Cycle (2008) 1.03
E-cadherin loss promotes the initiation of squamous cell carcinoma invasion through modulation of integrin-mediated adhesion. J Cell Sci (2006) 1.03
In vivo radioprotection by the fullerene nanoparticle DF-1 as assessed in a zebrafish model. Clin Cancer Res (2006) 1.02
Stretching type II collagen with optical tweezers. J Biomech (2004) 1.02
High-affinity binding of the NC1 domain of collagen VII to laminin 5 and collagen IV. Biochem Biophys Res Commun (2006) 1.02
Recessive COL6A2 C-globular missense mutations in Ullrich congenital muscular dystrophy: role of the C2a splice variant. J Biol Chem (2010) 1.01
Tumor necrosis factor-alpha and interleukin-1 antagonists alleviate inflammatory skin changes associated with epidermal growth factor receptor antibody therapy in mice. Cancer Res (2009) 0.99
A novel preclinical strategy for identifying cardiotoxic kinase inhibitors and mechanisms of cardiotoxicity. Circ Res (2011) 0.98
Malignant transformation of immortalized HaCaT keratinocytes through deregulated nuclear factor kappaB signaling. Cancer Res (2006) 0.98
Y-position cysteine substitution in type I collagen (alpha1(I) R888C/p.R1066C) is associated with osteogenesis imperfecta/Ehlers-Danlos syndrome phenotype. Hum Mutat (2007) 0.98
Coordinate control of cell cycle regulatory genes in zebrafish development tested by cyclin D1 knockdown with morpholino phosphorodiamidates and hydroxyprolyl-phosphono peptide nucleic acids. Nucleic Acids Res (2005) 0.97
Position of single amino acid substitutions in the collagen triple helix determines their effect on structure of collagen fibrils. J Struct Biol (2004) 0.96
Cell autonomous expression of inflammatory genes in biologically aged fibroblasts associated with elevated NF-kappaB activity. Immun Ageing (2008) 0.96
Differential expression of TRAIL and TRAIL receptors in allergic asthmatics following segmental antigen challenge: evidence for a role of TRAIL in eosinophil survival. J Immunol (2002) 0.96
Kuskokwim syndrome, a recessive congenital contracture disorder, extends the phenotype of FKBP10 mutations. Hum Mutat (2013) 0.95
Collagen fibril formation. A new target to limit fibrosis. J Biol Chem (2008) 0.94
Guilty by association: some collagen II mutants alter the formation of ECM as a result of atypical interaction with fibronectin. J Mol Biol (2005) 0.94
Nuclear factor kappaB inhibitors alleviate and the proteasome inhibitor PS-341 exacerbates radiation toxicity in zebrafish embryos. Mol Cancer Ther (2009) 0.93
Thermostability gradient in the collagen triple helix reveals its multi-domain structure. J Mol Biol (2004) 0.93
EGFR-dependent downregulation of Bim in epithelial cells requires MAPK and PKC-delta activities. Cancer Biol Ther (2006) 0.93
Procollagen VII self-assembly depends on site-specific interactions and is promoted by cleavage of the NC2 domain with procollagen C-proteinase. Biochemistry (2003) 0.91
Gene expression signatures modulated by epidermal growth factor receptor activation and their relationship to cetuximab resistance in head and neck squamous cell carcinoma. BMC Genomics (2012) 0.91
Differential regulation of p53 function by the N-terminal ΔNp53 and Δ113p53 isoforms in zebrafish embryos. BMC Dev Biol (2010) 0.91
Differential structural properties and expression patterns suggest functional significance for multiple mouse desmoglein 1 isoforms. Differentiation (2004) 0.89
Inhibition of cyclooxygenase-2 suppresses invasiveness of oral squamous cell carcinoma cell lines via down-regulation of matrix metalloproteinase-2 and CD44. Clin Exp Metastasis (2004) 0.89
Single amino acid substitutions in procollagen VII affect early stages of assembly of anchoring fibrils. J Biol Chem (2004) 0.89
Extracellular matrix protein 1 interacts with the domain III of fibulin-1C and 1D variants through its central tandem repeat 2. Biochem Biophys Res Commun (2005) 0.88
R992C (p.R1192C) Substitution in collagen II alters the structure of mutant molecules and induces the unfolded protein response. J Mol Biol (2009) 0.87
Human pancreatic carcinoma cells secrete bioactive interleukin-18 after treatment with 5-fluorouracil: implications for anti-tumor immune response. Cancer Biol Ther (2005) 0.87
Radioiodinated (I-125) monoclonal antibody 425 in the treatment of high grade glioma patients: ten-year synopsis of a novel treatment. Am J Clin Oncol (2002) 0.87
Testing the utility of rationally engineered recombinant collagen-like proteins for applications in tissue engineering. J Biomed Mater Res A (2006) 0.86
Design and development of masked therapeutic antibodies to limit off-target effects: application to anti-EGFR antibodies. Cancer Biol Ther (2009) 0.86
Molecular basis of organization of collagen fibrils. J Struct Biol (2006) 0.85
Procollagen with skipping of alpha 1(I) exon 41 has lower binding affinity for alpha 1(I) C-telopeptide, impaired in vitro fibrillogenesis, and altered fibril morphology. J Biol Chem (2001) 0.85
Site-specific interaction of bone morphogenetic protein 2 with procollagen II. Cytokine (2002) 0.85
Skeletal diseases caused by mutations that affect collagen structure and function. Int J Biochem Cell Biol (2013) 0.84
Identifying the SPARC binding sites on collagen I and procollagen I by atomic force microscopy. Anal Chem (2005) 0.84
Inhibition of glucocorticoid-induced apoptosis in 697 pre-B lymphocytes by the mineralocorticoid receptor N-terminal domain. J Biol Chem (2002) 0.83
A novel class of compounds with cutaneous wound healing properties. J Biomed Nanotechnol (2010) 0.83
TRAIL in the airways. Vitam Horm (2004) 0.83
Structural determinants of the selectivity of KTS-disintegrins for the alpha1beta1 integrin. FEBS Lett (2004) 0.82
Visualizing molecular polar order in tissues via electromechanical coupling. J Struct Biol (2012) 0.82
Prospects and limitations of the rational engineering of fibrillar collagens. Protein Sci (2003) 0.82
The triterpenoid RTA 408 is a robust mitigator of hematopoietic acute radiation syndrome in mice. Radiat Res (2015) 0.81
Engineering and characterization of the chimeric antibody that targets the C-terminal telopeptide of the α2 chain of human collagen I: a next step in the quest to reduce localized fibrosis. Connect Tissue Res (2013) 0.80
Persistence of intracellular and extracellular changes after incompletely suppressing expression of the R789C (p.R989C) and R992C (p.R1192C) collagen II mutants. Hum Mutat (2011) 0.80
Skinflammation and drug toxicity--a delicate balance. Sci Transl Med (2013) 0.80
Gene expression profiles as markers of aggressive disease-EGFR as a factor. Int J Radiat Oncol Biol Phys (2007) 0.80
Skin toxicity of targeted cancer agents: mechanisms and intervention. Future Oncol (2013) 0.79
Autoregulation of kinase dephosphorylation by ATP binding in AGC protein kinases. Cell Cycle (2012) 0.79
Antisense inhibition of cyclin D1 expression is equivalent to flavopiridol for radiosensitization of zebrafish embryos. Int J Radiat Oncol Biol Phys (2006) 0.78
Single amino acid substitutions in the C-terminus of collagen II alter its affinity for collagen IX. Biochem Biophys Res Commun (2005) 0.78
Fluorescent protein markers to tag collagenous proteins: the paradigm of procollagen VII. Biochem Biophys Res Commun (2009) 0.77
PET imaging of EGFR expression in nude mice bearing MDA-MB-468, a human breast adenocarcinoma. Nucl Med Commun (2011) 0.77
Biochemical composition and histologic structure of the forearm interosseous membrane. J Hand Surg Am (2003) 0.77
Endostatin binds nerve growth factor and thereby inhibits neurite outgrowth and neuronal migration in-vitro. Brain Res (2010) 0.77
Anchorless keratinocyte survival: an emerging pathogenic mechanism for squamous cell carcinoma in recessive dystrophic epidermolysis bullosa. Exp Dermatol (2007) 0.77
Testing the anti-fibrotic potential of the single-chain Fv antibody against the α2 C-terminal telopeptide of collagen I. Connect Tissue Res (2014) 0.77
Reducing the effects of intracellular accumulation of thermolabile collagen II mutants by increasing their thermostability in cell culture conditions. Biochem Biophys Res Commun (2010) 0.77
Therapeutic strategies for secondary hyperparathyroidism in dialysis patients. Ther Apher Dial (2006) 0.77