Published in Arthritis Rheum on May 01, 2012
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Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases. Hum Mol Genet (2013) 0.84
Analysis of the cartilage proteome from three different mouse models of genetic skeletal diseases reveals common and discrete disease signatures. Biol Open (2013) 0.80
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Missense mutations in the beta strands of the single A-domain of matrilin-3 result in multiple epiphyseal dysplasia. J Med Genet (2004) 1.30
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Decreased chondrocyte proliferation and dysregulated apoptosis in the cartilage growth plate are key features of a murine model of epiphyseal dysplasia caused by a matn3 mutation. Hum Mol Genet (2007) 1.18
Multiple epiphyseal dysplasia mutations in MATN3 cause misfolding of the A-domain and prevent secretion of mutant matrilin-3. Hum Mutat (2005) 1.12
Matrilin-3 forms disulfide-linked oligomers with matrilin-1 in bovine epiphyseal cartilage. J Biol Chem (1998) 1.10
Characterization of the matrilin coiled-coil domains reveals seven novel isoforms. J Biol Chem (2002) 1.05
Matrilin-3 mutations that cause chondrodysplasias interfere with protein trafficking while a mutation associated with hand osteoarthritis does not. J Med Genet (2005) 1.05
Abnormal collagen fibrils in cartilage of matrilin-1/matrilin-3-deficient mice. J Biol Chem (2007) 1.05
An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia. Cell Stress Chaperones (2010) 1.03
Novel and recurrent mutations clustered in the von Willebrand factor A domain of MATN3 in multiple epiphyseal dysplasia. Hum Mutat (2004) 1.02
Clinical and radiographic findings in multiple epiphyseal dysplasia caused by MATN3 mutations: description of 12 patients. Am J Med Genet A (2004) 0.98
Ablation of collagen IX and COMP disrupts epiphyseal cartilage architecture. Matrix Biol (2007) 0.98
Structural and functional characterization of recombinant matrilin-3 A-domain and implications for human genetic bone diseases. J Biol Chem (2007) 0.96
Genetic mouse models for the functional analysis of the perifibrillar components collagen IX, COMP and matrilin-3: Implications for growth cartilage differentiation and endochondral ossification. Histol Histopathol (2009) 0.95
A novel form of chondrocyte stress is triggered by a COMP mutation causing pseudoachondroplasia. Hum Mutat (2011) 0.93
Novel mutations in exon 2 of MATN3 affect residues within the alpha-helices of the A-domain and can result in the intracellular retention of mutant matrilin-3. Hum Mutat (2008) 0.88
Structural and functional investigations of Matrilin-1 A-domains reveal insights into their role in cartilage ECM assembly. J Biol Chem (2010) 0.87
Molecular properties of matrilin-3 isolated from human growth cartilage. Arch Biochem Biophys (2001) 0.84
Collagen IX: evidence for a structural association between NC4 domains in cartilage and a novel cleavage site in the alpha 1(IX) chain. Matrix Biol (1998) 0.80
Aberrant mucin assembly in mice causes endoplasmic reticulum stress and spontaneous inflammation resembling ulcerative colitis. PLoS Med (2008) 4.24
Endothelial function in HIV-infected persons. Clin Infect Dis (2006) 3.80
Collagens at a glance. J Cell Sci (2007) 2.26
Genetic diseases of connective tissues: cellular and extracellular effects of ECM mutations. Nat Rev Genet (2009) 2.03
Heterogeneity of airways mucus: variations in the amounts and glycoforms of the major oligomeric mucins MUC5AC and MUC5B. Biochem J (2002) 2.02
The expression and function of microRNAs in chondrogenesis and osteoarthritis. Arthritis Rheum (2011) 1.92
Muc5ac: a critical component mediating the rejection of enteric nematodes. J Exp Med (2011) 1.88
The circadian clock in murine chondrocytes regulates genes controlling key aspects of cartilage homeostasis. Arthritis Rheum (2013) 1.82
Bub1 maintains centromeric cohesion by activation of the spindle checkpoint. Dev Cell (2007) 1.72
Tracheobronchial air-liquid interface cell culture: a model for innate mucosal defense of the upper airways? Am J Physiol Lung Cell Mol Physiol (2008) 1.55
Targeted deletion of mek5 causes early embryonic death and defects in the extracellular signal-regulated kinase 5/myocyte enhancer factor 2 cell survival pathway. Mol Cell Biol (2005) 1.51
Mucin gene deficiency in mice impairs host resistance to an enteric parasitic infection. Gastroenterology (2010) 1.41
Reduced cell proliferation and increased apoptosis are significant pathological mechanisms in a murine model of mild pseudoachondroplasia resulting from a mutation in the C-terminal domain of COMP. Hum Mol Genet (2007) 1.35
On the origins of the extracellular matrix in vertebrates. Matrix Biol (2006) 1.35
Lineage tracing using matrilin-1 gene expression reveals that articular chondrocytes exist as the joint interzone forms. Dev Biol (2007) 1.30
MUC16 is produced in tracheal surface epithelium and submucosal glands and is present in secretions from normal human airway and cultured bronchial epithelial cells. Int J Biochem Cell Biol (2007) 1.30
MUC5B is the major mucin in the gel phase of sputum in chronic obstructive pulmonary disease. Am J Respir Crit Care Med (2008) 1.27
Superoxide dismutase downregulation in osteoarthritis progression and end-stage disease. Ann Rheum Dis (2010) 1.25
The integrins of the urochordate Ciona intestinalis provide novel insights into the molecular evolution of the vertebrate integrin family. BMC Evol Biol (2005) 1.23
Targeted induction of endoplasmic reticulum stress induces cartilage pathology. PLoS Genet (2009) 1.21
The unfolded protein response and its relevance to connective tissue diseases. Cell Tissue Res (2009) 1.20
Identification of molecular intermediates in the assembly pathway of the MUC5AC mucin. J Biol Chem (2004) 1.20
Decreased chondrocyte proliferation and dysregulated apoptosis in the cartilage growth plate are key features of a murine model of epiphyseal dysplasia caused by a matn3 mutation. Hum Mol Genet (2007) 1.18
A recessive skeletal dysplasia, SEMD aggrecan type, results from a missense mutation affecting the C-type lectin domain of aggrecan. Am J Hum Genet (2008) 1.17
Calcium-dependent protein interactions in MUC5B provide reversible cross-links in salivary mucus. J Biol Chem (2003) 1.14
COMP mutation screening as an aid for the clinical diagnosis and counselling of patients with a suspected diagnosis of pseudoachondroplasia or multiple epiphyseal dysplasia. Eur J Hum Genet (2005) 1.13
Multiple epiphyseal dysplasia mutations in MATN3 cause misfolding of the A-domain and prevent secretion of mutant matrilin-3. Hum Mutat (2005) 1.12
Targeted deletion of the mitogen-activated protein kinase kinase 4 gene in the nervous system causes severe brain developmental defects and premature death. Mol Cell Biol (2007) 1.11
Ex vivo sputum analysis reveals impairment of protease-dependent mucus degradation by plasma proteins in acute asthma. Am J Respir Crit Care Med (2009) 1.11
Collagen XXVII is developmentally regulated and forms thin fibrillar structures distinct from those of classical vertebrate fibrillar collagens. J Biol Chem (2007) 1.10
HtrA1 inhibits mineral deposition by osteoblasts: requirement for the protease and PDZ domains. J Biol Chem (2007) 1.03
An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia. Cell Stress Chaperones (2010) 1.03
Particle tracking microrheology of purified gastrointestinal mucins. Biopolymers (2014) 1.02
COL10A1 nonsense and frame-shift mutations have a gain-of-function effect on the growth plate in human and mouse metaphyseal chondrodysplasia type Schmid. Hum Mol Genet (2007) 1.02
The evolution of the vertebrate metzincins; insights from Ciona intestinalis and Danio rerio. BMC Evol Biol (2007) 1.02
Recurrent dominant mutations affecting two adjacent residues in the motor domain of the monomeric kinesin KIF22 result in skeletal dysplasia and joint laxity. Am J Hum Genet (2011) 1.02
Proteomic analysis of mouse growth plate cartilage. Proteomics (2006) 1.01
A mouse model offers novel insights into the myopathy and tendinopathy often associated with pseudoachondroplasia and multiple epiphyseal dysplasia. Hum Mol Genet (2010) 1.00
Transcriptional profiling of chondrodysplasia growth plate cartilage reveals adaptive ER-stress networks that allow survival but disrupt hypertrophy. PLoS One (2011) 1.00
Serine protease(s) secreted by the nematode Trichuris muris degrade the mucus barrier. PLoS Negl Trop Dis (2012) 0.99
Concentrated solutions of salivary MUC5B mucin do not replicate the gel-forming properties of saliva. Biochem J (2002) 0.99
Clinical and radiographic findings in multiple epiphyseal dysplasia caused by MATN3 mutations: description of 12 patients. Am J Med Genet A (2004) 0.98
Mutations in the known genes are not the major cause of MED; distinctive phenotypic entities among patients with no identified mutations. Eur J Hum Genet (2005) 0.96
Structural and functional characterization of recombinant matrilin-3 A-domain and implications for human genetic bone diseases. J Biol Chem (2007) 0.96
The collagens of hydra provide insight into the evolution of metazoan extracellular matrices. J Biol Chem (2007) 0.96
Identification of multiple integrin beta1 homologs in zebrafish (Danio rerio). BMC Cell Biol (2006) 0.95
The characterisation of six ADAMTS proteases in the basal chordate Ciona intestinalis provides new insights into the vertebrate ADAMTS family. Int J Biochem Cell Biol (2005) 0.95
Proteomic analysis of polymeric salivary mucins: no evidence for MUC19 in human saliva. Biochem J (2008) 0.94
Col2a1 lineage tracing reveals that the meniscus of the knee joint has a complex cellular origin. J Anat (2008) 0.94
A novel form of chondrocyte stress is triggered by a COMP mutation causing pseudoachondroplasia. Hum Mutat (2011) 0.93
Receptor tyrosine kinase Axl modulates the osteogenic differentiation of pericytes. Circ Res (2003) 0.93
A new role for mucins in immunity: insights from gastrointestinal nematode infection. Int J Biochem Cell Biol (2012) 0.93
Type IX collagen gene mutations can result in multiple epiphyseal dysplasia that is associated with osteochondritis dissecans and a mild myopathy. Am J Med Genet A (2010) 0.92
Electronic memorandum decreases unnecessary antimicrobial use for asymptomatic bacteriuria and culture-negative pyuria. Infect Control Hosp Epidemiol (2011) 0.90
Collagen XXVII organises the pericellular matrix in the growth plate. PLoS One (2011) 0.90
An enhancer complex confers both high-level and cell-specific expression of the human type X collagen gene. FEBS Lett (2002) 0.90
Utility of cystatin C to monitor renal function in Duchenne muscular dystrophy. Muscle Nerve (2009) 0.89
Novel mutations in exon 2 of MATN3 affect residues within the alpha-helices of the A-domain and can result in the intracellular retention of mutant matrilin-3. Hum Mutat (2008) 0.88
Identification of salivary mucin MUC7 binding proteins from Streptococcus gordonii. BMC Microbiol (2009) 0.88
Structural and functional investigations of Matrilin-1 A-domains reveal insights into their role in cartilage ECM assembly. J Biol Chem (2010) 0.87
Preselection of cases through expert clinical and radiological review significantly increases mutation detection rate in multiple epiphyseal dysplasia. Eur J Hum Genet (2006) 0.87
A novel role for Gtb1p in glucose trimming of N-linked glycans. Glycobiology (2009) 0.87
A secretory cell type develops alongside multiciliated cells, ionocytes and goblet cells, and provides a protective, anti-infective function in the frog embryonic mucociliary epidermis. Development (2014) 0.86
Muc5b and Muc5ac are the major oligomeric mucins in equine airway mucus. Am J Physiol Lung Cell Mol Physiol (2007) 0.85
Back to basics--how the evolution of the extracellular matrix underpinned vertebrate evolution. Int J Exp Pathol (2009) 0.84
Review: clinical variability and genetic heterogeneity in multiple epiphyseal dysplasia. Pediatr Pathol Mol Med (2003) 0.84
Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases. Hum Mol Genet (2013) 0.84
Partial characterisation of high-molecular weight glycoconjugates in the trail mucus of the freshwater pond snail Lymnaea stagnalis. Comp Biochem Physiol B Biochem Mol Biol (2004) 0.82
A novel transgenic mouse model of growth plate dysplasia reveals that decreased chondrocyte proliferation due to chronic ER stress is a key factor in reduced bone growth. Dis Model Mech (2013) 0.81
Expression and secretion of Aspergillus fumigatus proteases are regulated in response to different protein substrates. Fungal Biol (2012) 0.81
Analysis of the cartilage proteome from three different mouse models of genetic skeletal diseases reveals common and discrete disease signatures. Biol Open (2013) 0.80
Desulfurization of mucin by Pseudomonas aeruginosa: influence of sulfate in the lungs of cystic fibrosis patients. J Med Microbiol (2012) 0.80
Skeletal dysplasias associated with mild myopathy-a clinical and molecular review. J Biomed Biotechnol (2010) 0.80
TGF-β₂ decreases baseline and IL-13-stimulated mucin production by primary human bronchial epithelial cells. Exp Lung Res (2012) 0.78
Hypertrophic chondrocytes have a limited capacity to cope with increases in endoplasmic reticulum stress without triggering the unfolded protein response. J Histochem Cytochem (2012) 0.78
A combined small-angle X-ray and neutron scattering study of the structure of purified soluble gastrointestinal mucins. Biopolymers (2014) 0.78