Disease: Exostoses- multiple- type 1
- A mouse model of chondrocyte-specific somatic mutation reveals a role for Ext1 loss of heterozygosity in multiple hereditary exostoses
- A mouse model of osteochondromagenesis from clonal inactivation of Ext1 in chondrocytes
- A mutation in <em>SLC20A2</em> (c.C1849T) promotes proliferation while inhibiting hypertrophic differentiation in ATDC5 chondrocytes
- A New Classification System for Forearm Deformities Caused by Hereditary Multiple Osteochondromas
- A novel splice mutation induces exon skipping of the EXT1 gene in patients with hereditary multiple exostoses
- A patient with novel mutations causing MEN1 and hereditary multiple osteochondroma
- A single-base change in the tyrosine kinase II domain of ovine FGFR3 causes hereditary chondrodysplasia in sheep
- A splice mutation and mRNA decay of EXT2 provoke hereditary multiple exostoses
- A systematic review of genetic skeletal disorders reported in Chinese biomedical journals between 1978 and 2012
- Acroform type of enchondromatosis associated with severe vertebral involvement and facial dysmorphism in a boy with a new variant of enchondromatosis type I1 of Spranger: case report and a review of the literature
- An analysis of osteoporosis in patients with hereditary multiple exostoses
- An Evaluation of Forearm Deformities in Hereditary Multiple Exostoses: Factors Associated With Radial Head Dislocation and Comprehensive Classification
- An extended outbreak of congenital chondrodysplasia in calves in South East Australia
- An interstitial deletion of 8q23.3-q24.22 associated with Langer-Giedion syndrome, Cornelia de Lange syndrome and epilepsy
- Annular pancreas in Trichorhinophalangeal syndrome type II with 8q23.3-q24.12 interstitial deletion
- Atypical teratoid/rhabdoid tumor with hereditary multiple exostoses in an 18-year-old male: A case report
- Carriers of loss-of-function mutations in EXT display impaired pancreatic beta-cell reserve due to smaller pancreas volume
- Cartilage-specific overexpression of ERRγ results in Chondrodysplasia and reduced chondrocyte proliferation
- Clinicopathologic features of osteochondroma with malignant transformation
- Cytokines in bone diseases. Genetic defects of PTH/PTHrP receptor in chondrodysplasia
- Defective chondrocyte proliferation and differentiation in osteochondromas of MHE patients
- Differentiation-induced loss of heparan sulfate in human exostosis derived chondrocytes
- Disruption of gastrulation and heparan sulfate biosynthesis in EXT1-deficient mice
- Distinct osmoregulatory responses to sodium loading in patients with altered glycosaminoglycan structure: a randomized cross-over trial
- Distraction osteogenesis at the proximal third of the ulna for the treatment of Masada type I/IIb deformities in children with hereditary multiple exostoses: a retrospective review of twenty cases
- Effect of osteochondroma location on forearm deformity in patients with multiple hereditary osteochondromatosis
- Embryonic fibroblasts with a gene trap mutation in Ext1 produce short heparan sulfate chains
- Etiological point mutations in the hereditary multiple exostoses gene EXT1: a functional analysis of heparan sulfate polymerase activity
- Evidence of genetic underexpression in chorionic villi samples of euploid fetuses with increased nuchal translucency at 10-11 weeks' gestation
- Exostoses, enchondromatosis and metachondromatosis; diagnosis and management
- Expanding the clinical and molecular features of trichorhino- phalangeal syndrome with a novel variant
- Expanding the phenotypic spectrum of variants in PDE4D/PRKAR1A: from acrodysostosis to acroscyphodysplasia
- EXT 1 gene mutation induces chondrocyte cytoskeletal abnormalities and defective collagen expression in the exostoses
- EXTL2, a member of the EXT family of tumor suppressors, controls glycosaminoglycan biosynthesis in a xylose kinase-dependent manner
- Factors influencing postoperative bleeding after dental extraction in older adult patients receiving anticoagulation therapy
- Familial nephropathy and multiple exostoses with exostosin-1 (EXT1) gene mutation
- Forearm Hereditary Multiple Exostosis: A Retrospective Case Series Study
- Functional Requirements for Heparan Sulfate Biosynthesis in Morphogenesis and Nervous System Development in C. elegans
- Gradual ulnar lengthening by an Ilizarov ring fixator for correction of Masada IIb forearm deformity without tumor excision in hereditary multiple exostosis: preliminary results
- Gradual ulnar lengthening in Masada type I/IIb deformity in patients with hereditary multiple osteochondromas: a retrospective study with a mean follow-up of 4.2 years
- Haploinsufficiency of EXT1 and Heparan Sulphate Deficiency Associated with Hereditary Multiple Exostoses in a Pakistani Family
- Heparan sulfate antagonism alters bone morphogenetic protein signaling and receptor dynamics, suggesting a mechanism in hereditary multiple exostoses
- Heparan sulfate in skeletal development, growth, and pathology: the case of hereditary multiple exostoses
- Herpes simplex virus: discovering the link between heparan sulphate and hereditary bone tumours
- Hinge positioning method of Ilizarov apparatus in correcting radial head luxation caused by multiple hereditary exostoses
- Hip-Spine Syndrome: Is There an Association Between Markers for Cam Deformity and Osteoarthritis of the Lumbar Spine?
- HSPG-deficient zebrafish uncovers dental aspect of multiple osteochondromas
- Identification of mutation in a candidate gene for hereditary multiple exostoses type II
- Identification of risk factors affecting bone formation in gradual ulnar lengthening in children with hereditary multiple exostoses: A retrospective study
- Identification of six novel PTH1R mutations in families with a history of primary failure of tooth eruption
- Intraosseous atypical chondroid tumor or chondrosarcoma grade 1 in patients with multiple osteochondromas
- Komrower Lecture. Congenital disorders of glycosylation (CDG): it's all in it!
- Langer-Giedion syndrome with 8q23.1-q24.12 deletion diagnosed by comparative genomic hybridization
- Loss of function in heparan sulfate elongation genes EXT1 and EXT 2 results in improved nitric oxide bioavailability and endothelial function
- Loss-of-function mutations in PTPN11 cause metachondromatosis, but not Ollier disease or Maffucci syndrome
- Lower limb deformities and limb length discrepancies in hereditary multiple exostoses
- Management of forearm deformities with ulnar shortening more than 15 mm caused by hereditary multiple osteochondromas
- Metaphyseal dysplasia: a new autosomal dominant type in a large German kindred
- Mixed Phenotype of Langer-Giedion's and Cornelia de Lange's Syndromes in an 8q23.3-q24.1 Microdeletion without <em>TRPS1</em> Deletion
- Mixed Phenotype of Langer-Giedion's and Cornelia de Lange's Syndromes in an 8q23.3-q24.1 Microdeletion without TRPS1 Deletion
- Modified ulnar lengthening for correction of the Masada type 2 forearm deformity in hereditary multiple exostosis
- Morphological classification for prediction of malignant transformation in multiple exostoses
- Mutation frequencies of EXT1 and EXT2 in 43 Japanese families with hereditary multiple exostoses
- Mutation in the heparan sulfate biosynthesis enzyme EXT1 influences growth factor signaling and fibroblast interactions with the extracellular matrix
- Mutational Analysis of <em>EXT1</em>in a Chinese Family Affected by Hereditary Multiple Osteochondroma
- Mutational Analysis of EXT1in a Chinese Family Affected by Hereditary Multiple Osteochondroma
- Mutational spectrum and clinical signatures in 114 families with hereditary multiple osteochondromas: insights into molecular properties of selected exostosin variants
- Natural History and Characteristics of Hand Exostoses in Multiple Hereditary Exostoses
- Neutral sphingomyelinase (SMPD3) deficiency causes a novel form of chondrodysplasia and dwarfism that is rescued by Col2A1-driven smpd3 transgene expression
- Non-ossifying fibroma with a pathologic fracture in a 12-year-old girl with tricho-rhino-phalangeal syndrome: a case report
- Not All Osteophytes Are Located on the Right Side of the Vertebrae in Diffuse Idiopathic Skeletal Hyperostosis: A Quantitative Analysis in Relation to the Position of Aorta
- Older age and multi-joint external fixator are two risk factors of complications in ulnar lengthening in children with hereditary multiple exostosis
- Osteochondroma of acromioclavicular joint
- Parathyroid hormone 1 receptor: insights into structure and function
- Partial rescue of PTH/PTHrP receptor knockout mice by targeted expression of the Jansen transgene
- Recent studies on the biological action of parathyroid hormone (PTH)-related peptide (PTHrP) and PTH/PTHrP receptor in cartilage and bone
- Recessive mutations in PTHR1 cause contrasting skeletal dysplasias in Eiken and Blomstrand syndromes
- Reconstruction of forearm deformities in multiple cartilaginous exostoses
- Reduced chondrocyte proliferation and chondrodysplasia in mice lacking the integrin-linked kinase in chondrocytes
- Regulation of zebrafish skeletogenesis by ext2/dackel and papst1/pinscher
- Short rib-polydactyly syndrome: lethal chondrodysplasia associated with brain malformations in a 35-week-gestation infant
- SHP2 Regulates the Osteogenic Fate of Growth Plate Hypertrophic Chondrocytes
- Signaling systems affecting the severity of multiple osteochondromas
- Single-Bone Forearm as a Salvage Procedure in Recalcitrant Pediatric Forearm Pathologies
- Tackling the number-one killer in my community
- The cyclin D1 and cyclin A genes are targets of activated PTH/PTHrP receptors in Jansen's metaphyseal chondrodysplasia
- The evolving therapeutic landscape of genetic skeletal disorders
- The link between heparan sulfate and hereditary bone disease: finding a function for the EXT family of putative tumor suppressor proteins
- The One-Bone Forearm in Children: Surgical Technique and a Retrospective Review of Outcomes
- The short-lived exostosis induced surgically versus the lasting genetic hereditary multiple exostoses
- The type 2 diabetes associated rs7903146 T allele within TCF7L2 is significantly under-represented in Hereditary Multiple Exostoses: insights into pathogenesis
- Total hip arthroplasty in hereditary multiple exostosis patients: literature review and evaluation of 10 cases
- Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report
- Trichorhinophalangeal syndrome type 1: A case report with literature review
- Ulnar Distraction Osteogenesis in the Treatment of Forearm Deformities in Children With Multiple Hereditary Exostoses
- Usefulness of fetal autopsy in the diagnosis of blomstrand chondrodysplasia: a report of three cases
- Variations in the HHEX gene are associated with increased risk of type 2 diabetes in the Japanese population
- Whole-exome sequencing identifies a novel mutation of SLC20A2 (c.C1849T) as a possible cause of hereditary multiple exostoses in a Chinese family
- Whole‑exome sequencing identifies a novel mutation of SLC20A2 (c.C1849T) as a possible cause of hereditary multiple exostoses in a Chinese family
- Y-position collagen II mutation disrupts cartilage formation and skeletal development in a transgenic mouse model of spondyloepiphyseal dysplasia