Disease: Exostoses- multiple- type 2
- A collaborative approach to the diagnosis of a lethal short limb skeletal dysplasia
- A direct interaction between EXT proteins and glycosyltransferases is defective in hereditary multiple exostoses
- A family with limb girdle muscular dystrophy type 1B and multiple 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 Intronic Splicing Mutation in the <em>EXT2</em> Gene of a Chinese Family with Multiple Osteochondroma
- A Novel Intronic Splicing Mutation in the EXT2 Gene of a Chinese Family with Multiple Osteochondroma
- A novel mutation substituting tryptophan with arginine in the carboxyl-terminal, non-collagenous domain of collagen X in a case of Schmid metaphyseal chondrodysplasia
- A patient with novel mutations causing MEN1 and hereditary multiple osteochondroma
- A splice mutation and mRNA decay of EXT2 provoke hereditary multiple exostoses
- Acroscyphodysplasia as a phenotypic variation of pseudohypoparathyroidism and acrodysostosis type 2
- 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
- Annular pancreas in Trichorhinophalangeal syndrome type II with 8q23.3-q24.12 interstitial deletion
- Carriers of loss-of-function mutations in EXT display impaired pancreatic beta-cell reserve due to smaller pancreas volume
- Clear cell chondrosarcoma in association with niemann-pick disease
- Clinical and Functional Outcomes of Ulnar Lengthening in the Treatment of Masada Type I Forearm Deformities in Hereditary Multiple Osteochondromas
- Clinical and molecular characterization of a patient with Langer-Giedion syndrome and mosaic del(8)(q22.3q24.13)
- Clinicopathologic features of osteochondroma with malignant transformation
- Deep dental phenotyping and a novel FAM20A variant in patients with amelogenesis imperfecta type IG
- Demonstration of a novel gene DEXT3 of Drosophila melanogaster as the essential N-acetylglucosamine transferase in the heparan sulfate biosynthesis: chain initiation and elongation
- Differentiation-induced loss of heparan sulfate in human exostosis derived chondrocytes
- Disrupted expression of matrix genes in the growth plate of the mouse cartilage matrix deficiency (cmd) mutant
- Disruption of gastrulation and heparan sulfate biosynthesis in EXT1-deficient mice
- 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
- Elevated hypertrophy, growth plate maturation, glycosaminoglycan deposition, and exostosis formation in the Hspg2 exon 3 null mouse intervertebral disc
- 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
- 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
- EXT-mutation analysis and loss of heterozygosity in sporadic and hereditary osteochondromas and secondary chondrosarcomas
- 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
- Filamin-interacting proteins, Cfm1 and Cfm2, are essential for the formation of cartilaginous skeletal elements
- 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
- 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
- Insight into Schmid metaphyseal chondrodysplasia from the crystal structure of the collagen X NC1 domain trimer
- 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: a Rare Case Report
- Loss of function in heparan sulfate elongation genes EXT1 and EXT 2 results in improved nitric oxide bioavailability and endothelial function
- Loss of β-catenin induces multifocal periosteal chondroma-like masses in mice
- 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
- Misfolding of collagen X chains harboring Schmid metaphyseal chondrodysplasia mutations results in aberrant disulfide bond formation, intracellular retention, and activation of the unfolded protein response
- Mixed Phenotype of Langer-Giedion's and Cornelia de Lange's Syndromes in an 8q23.3-q24.1 Microdeletion without <em>TRPS1</em> Deletion
- Modified ulnar lengthening for correction of the Masada type 2 forearm deformity in hereditary multiple exostosis
- Multiple Cartilaginous Exostoses in a Cat
- Multiple osteochondroma of the hand: initial and long-term follow-up study
- Multiple Skeletal Deformities in a Middle-Aged Man
- 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
- Mutations in a new gene, encoding a zinc-finger protein, cause tricho-rhino-phalangeal syndrome type I
- Natural History and Characteristics of Hand Exostoses in Multiple Hereditary Exostoses
- New Insights into Potocki-Shaffer Syndrome: Report of Two Novel Cases and Literature Review
- Phenotypic and genotypic overlap between atelosteogenesis type 2 and diastrophic dysplasia
- Positional cloning of the putative gene responsible for transient abnormal myelopoiesis and that for multiple cartilaginous exostoses
- Postzygotic mosaicism of a novel PTPN11 mutation in monozygotic twins discordant for metachondromatosis
- Proteoglycan sulfation in cartilage and cell cultures from patients with sulfate transporter chondrodysplasias: relationship to clinical severity and indications on the role of intracellular sulfate production
- Recent studies on the biological action of parathyroid hormone (PTH)-related peptide (PTHrP) and PTH/PTHrP receptor in cartilage and bone
- Reconstruction of forearm deformities in multiple cartilaginous exostoses
- Regulation of zebrafish skeletogenesis by ext2/dackel and papst1/pinscher
- Retinoid roles and action in skeletal development and growth provide the rationale for an ongoing heterotopic ossification prevention trial
- Short rib-polydactyly syndrome: lethal chondrodysplasia associated with brain malformations in a 35-week-gestation infant
- SHP2 regulates chondrocyte terminal differentiation, growth plate architecture and skeletal cell fates
- Single-Bone Forearm as a Salvage Procedure in Recalcitrant Pediatric Forearm Pathologies
- The cyclin D1 and cyclin A genes are targets of activated PTH/PTHrP receptors in Jansen's metaphyseal chondrodysplasia
- 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 putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate
- The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate
- 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
- Trichorhinophalangeal syndrome type 1: A case report with literature review
- Trichorhinophalangeal syndrome, type II (Langer-Giedion syndrome)
- Type I osteogenesis imperfecta and multiple osteochondromas in the same child
- Ulnar Distraction Osteogenesis in the Treatment of Forearm Deformities in Children With Multiple Hereditary Exostoses
- Ulnar Lengthening/Reconstruction of Interosseous Membrane in Treatment of Osteochondroma
- Variations in the HHEX gene are associated with increased risk of type 2 diabetes in the Japanese population
- Vascular anomalies in Proteus syndrome
- Vitrified blastocysts from Preimplantation Genetic Diagnosis (PGD) as a source for human Embryonic Stem Cell (hESC) derivation
- 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