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https://www.readbyqxmd.com/read/28918311/structural-basis-for-the-potent-and-selective-binding-of-ldn-212854-to-the-bmp-receptor-kinase-alk2
#1
Eleanor Williams, Alex N Bullock
Individuals with the rare developmental disorder fibrodysplasia ossificans progressiva (FOP) experience disabling heterotopic ossification caused by a gain of function mutation in the intracellular region of the BMP type I receptor kinase ALK2, encoded by the gene ACVR1. Small molecule BMP type I receptor inhibitors that block this ossification in FOP mouse models have been derived from the pyrazolo[1,5-a]pyrimidine scaffold of dorsomorphin. While the first derivative LDN-193189 exhibited pan inhibition of BMP receptors, the more recent compound LDN-212854 has shown increased selectivity for ALK2...
September 12, 2017: Bone
https://www.readbyqxmd.com/read/28866367/the-fop-connection-registry-design-of-an-international-patient-sponsored-registry-for-fibrodysplasia-ossificans-progressiva
#2
Neal Mantick, Eric Bachman, Genevieve Baujat, Matt Brown, Oliver Collins, Carmen De Cunto, Patricia Delai, Marelise Eekhoff, Roger Zum Felde, Donna Roy Grogan, Nobuhiko Haga, Edward Hsiao, Sharon Kantanie, Frederick Kaplan, Richard Keen, Jelena Milosevic, Rolf Morhart, Robert Pignolo, Xiaobing Qian, Maja di Rocco, Christiaan Scott, Adam Sherman, Marin Wallace, Nicky Williams, Keqin Zhang, Betsy Bogard
The Fibrodysplasia Ossificans Progressiva (FOP) Connection Registry is an international, voluntary, observational study that directly captures demographic and disease information initially from patients with FOP (the patient portal) and in the near future from treating physicians (the physician portal) via a secure web-based tool. It was launched by the International FOP Association (IFOPA) with a guiding vision to develop and manage one unified, global, and coordinated Registry allowing the assembly of the most comprehensive data on FOP...
August 30, 2017: Bone
https://www.readbyqxmd.com/read/28855331/drosophila-nociceptive-sensitization-requires-bmp-signaling-via-the-canonical-smad-pathway
#3
Taylor L Follansbee, Kayla J Gjelsvik, Courtney L Brann, Aidan L McParland, Colin A Longhurst, Michael J Galko, Geoffrey K Ganter
Nociceptive sensitization is a common feature in chronic pain, but its basic cellular mechanisms are only partially understood. The present study used the Drosophila melanogaster model system and a candidate gene approach to identify novel components required for modulation of an injury-induced nociceptive sensitization pathway presumably downstream of Hedgehog. This study demonstrates that RNAi silencing of a member of the Bone Morphogenetic Protein (BMP) signaling pathway, Decapentaplegic (Dpp), specifically in the Class IV multidendritic nociceptive neuron, significantly attenuated ultraviolet injury-induced sensitization...
August 30, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28851540/mast-cell-inhibition-as-a-therapeutic-approach-in-fibrodysplasia-ossificans-progressiva-fop
#4
Tracy A Brennan, Carter M Lindborg, Christian R Bergbauer, Haitao Wang, Frederick S Kaplan, Robert J Pignolo
BACKGROUND: Episodic flare-ups of fibrodysplasia ossificans progressiva (FOP) are characterized clinically by severe, often posttraumatic, connective tissue swelling and intramuscular edema, followed histologically by an intense and highly angiogenic fibroproliferative reaction. This early inflammatory and angiogenic fibroproliferative response is accompanied by the presence of abundant mast cells far in excess of other reported myopathies. RESULTS: Using an injury-induced, constitutively-active transgenic mouse model of FOP we show that mast cell inhibition by cromolyn, but not aprepitant, results in a dramatic reduction of heterotopic ossification...
August 26, 2017: Bone
https://www.readbyqxmd.com/read/28826842/retinoid-roles-and-action-in-skeletal-development-and-growth-provide-the-rationale-for-an-ongoing-heterotopic-ossification-prevention-trial
#5
Maurizio Pacifici
The majority of skeletal elements develop via endochondral ossification. This process starts with formation of mesenchymal cell condensations at prescribed sites and times in the early embryo and is followed by chondrogenesis, growth plate cartilage maturation and hypertrophy, and replacement of cartilage with bone and marrow. This complex stepwise process is reactivated and recapitulated in physiologic conditions such as fracture repair, but can occur extraskeletally in pathologies including heterotopic ossification (HO), Ossification of the Posterior Longitudinal Ligament (OPLL) and Hereditary Multiple Exostoses (HME)...
August 18, 2017: Bone
https://www.readbyqxmd.com/read/28826841/-18f-naf-pet-ct-scan-as-an-early-marker-of-heterotopic-ossification-in-fibrodysplasia-ossificans-progressiva
#6
E Marelise W Eekhoff, Esmée Botman, J Coen Netelenbos, Pim de Graaf, Nathalie Bravenboer, Dimitra Micha, Gerard Pals, Teun J de Vries, T Ton Schoenmaker, Max Hoebink, Adriaan Lammertsma, Pieter Raijmakers
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease with a progressive course characterized by episodically local flare-ups, which often but not always leads to heterotopic bone formation (HO). Recently, we showed that [18F]NaF PET/CT may be the first tool to monitor progression of a posttraumatic flare-up leading to new HO, which was demonstrated in a patient with FOP who underwent a maxillofacial surgery. This paper evaluates [18F]NaF PET/CT as a marker of FOP disease activity, comparing its use with other imaging modalities known in literature...
August 18, 2017: Bone
https://www.readbyqxmd.com/read/28822792/imaging-assessment-of-fibrodysplasia-ossificans-progressiva-qualitative-quantitative-and-questionable
#7
Mona Al Mukaddam, Chamith S Rajapakse, Robert J Pignolo, Frederick S Kaplan, Stacy E Smith
Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare autosomal dominant genetic disorder of heterotopic ossification (HO) characterized by skeletal anomalies and episodic soft tissue swelling (flare-ups) that can transform into heterotopic bone. The progressive development of heterotopic bone and progressive arthropathy leads to significant limitation of mobility. This paper will review various imaging modalities used in evaluating episodic soft tissue swelling (flare-ups), heterotopic bone and skeletal anomalies...
August 16, 2017: Bone
https://www.readbyqxmd.com/read/28822791/acute-unilateral-hip-pain-in-fibrodysplasia-ossificans-progressiva-fop
#8
Frederick S Kaplan, Mona Al Mukaddam, Robert J Pignolo
BACKGROUND: Flare-ups of the hips are among the most feared and disabling complications of fibrodysplasia ossificans progressiva (FOP) and are poorly understood. In order to better understand the nature of hip flare-ups in FOP, we evaluated 25 consecutive individuals with classic FOP (14 males, 11 females; 3-56years old, median age, 17years old) who presented with acute unilateral hip pain. RESULTS: All 25 individuals were suspected of having a flare-up of the hip based on clinical history and a favorable response to a four day course of high-dose oral prednisone...
August 16, 2017: Bone
https://www.readbyqxmd.com/read/28797219/unresolving-trismus-following-third-molar-surgery-report-of-a-case-of-fibrodysplasia-ossificans-progressiva-with-review-of-literature
#9
B R Rajanikanth, Kavitha Prasad, K Vineeth, Sushma M N Sonale, Khuteja Al-Kubra
BACKGROUND: Trismus is a problem commonly encountered by the dental practitioner. It has a number of potential causes, and its treatment will depend on the cause. However, there are very few reports of trismus due to fibrodysplasia ossificans progressiva (FOP) following third molar surgery. CLINICAL PRESENTATION: FOP is a rare human genetic disorder with characteristic clinical features like progressive formation of extraskeletal bone or heterotopic ossification and congenital malformation of the great toes...
August 11, 2017: Cranio: the Journal of Craniomandibular Practice
https://www.readbyqxmd.com/read/28795074/unsupervised-exercise-induced-myositis-ossificans-in-the-brachialis-muscle-of-a-young-healthy-male-a-case-report
#10
Nikiforos Galanis, Chara Stavraka, Evdoxia Valavani, John Kirkos
No abstract text is available yet for this article.
July 2017: Orthopaedic Journal of Sports Medicine
https://www.readbyqxmd.com/read/28782882/the-expansion-of-heterotopic-bone-in-fibrodysplasia-ossificans-progressiva-is-activin-a-dependent
#11
Jaymin Upadhyay, LiQin Xie, Lily Huang, Nanditha Das, Rachel C Stewart, Morgan C Lyon, Keryn Palmer, Saathyaki Rajamani, Chris Graul, Merryl Lobo, Tyler J Wellman, Edward J Soares, Matthew D Silva, Jacob Hesterman, Lili Wang, Xialing Wen, Xiaobing Qian, Kalyan Nannuru, Vincent Idone, Andrew J Murphy, Aris N Economides, Sarah J Hatsell
Fibrodysplasia Ossificans Progressiva (FOP) is a rare autosomal dominant disorder that is characterized by episodic yet cumulative heterotopic ossification (HO) in skeletal muscles, tendons, and ligaments over a patient's lifetime. FOP is caused by missense mutations in the type I Bone Morphogenetic Protein (BMP) receptor ACVR1. We have determined that the formation of heterotopic bone in FOP requires activation of mutant ACVR1 by Activin A, in part by showing that prophylactic inhibition of Activin A blocks HO in a mouse model of FOP...
August 7, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28780628/surgical-excision-of-symptomatic-mature-posttraumatic-myositis-ossificans-characteristics-and-outcomes-in-32-athletes
#12
Sakari Orava, Juha-Jaakko Sinikumpu, Janne Sarimo, Lasse Lempainen, Gideon Mann, Iftach Hetsroni
PURPOSE: The purpose of this study was to report characteristics and outcomes of surgical excision of symptomatic mature posttraumatic myositis ossificans in adult athletes. The hypothesis was that surgical excision of the ossified mass in these circumstances can effectively relief symptoms and result in return to high-level sports with minimal postoperative complications. METHODS: All operations involving excision of posttraumatic heterotopic ossifications performed between 1987 and 2015 were reviewed...
August 5, 2017: Knee Surgery, Sports Traumatology, Arthroscopy: Official Journal of the ESSKA
https://www.readbyqxmd.com/read/28780023/shared-acvr1-mutations-in-fop-and-dipg-opportunities-and-challenges-in-extending-biological-and-clinical-implications-across-rare-diseases
#13
Harry J Han, Payal Jain, Adam C Resnick
Gain-of-function mutations in the Type I Bone Morphogenic Protein (BMP) receptor ACVR1 have been identified in two diseases: Fibrodysplasia Ossificans Progressiva (FOP), a rare autosomal dominant disorder characterized by genetically driven heterotopic ossification, and in 20-25% of Diffuse Intrinsic Pontine Gliomas (DIPGs), a pediatric brain tumor with no effective therapies and dismal median survival. While the ACVR1 mutation is causal for FOP, its role in DIPG tumor biology remains under active investigation...
August 2, 2017: Bone
https://www.readbyqxmd.com/read/28758906/activin-a-enhances-mtor-signaling-to-promote-aberrant-chondrogenesis-in-fibrodysplasia-ossificans-progressiva
#14
Kyosuke Hino, Kazuhiko Horigome, Megumi Nishio, Shingo Komura, Sanae Nagata, Chengzhu Zhao, Yonghui Jin, Koichi Kawakami, Yasuhiro Yamada, Akira Ohta, Junya Toguchida, Makoto Ikeya
Fibrodysplasia ossificans progressiva (FOP) is a rare and intractable disease characterized by extraskeletal bone formation through endochondral ossification. Patients with FOP harbor point mutations in ACVR1, a type I receptor for BMPs. Although mutated ACVR1 (FOP-ACVR1) has been shown to render hyperactivity in BMP signaling, we and others have uncovered a mechanism by which FOP-ACVR1 mistransduces BMP signaling in response to Activin-A, a molecule that normally transduces TGF-β signaling. Although Activin-A evokes enhanced chondrogenesis in vitro and heterotopic ossification (HO) in vivo, the underlying mechanisms have yet to be revealed...
September 1, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28756986/extraskeletal-aneurysmal-bone-cyst-report-of-a-case-and-review-of-the-literature
#15
Liurka V Lopez, Michael G Rodriguez, Gene P Siegal, Shi Wei
Aneurysmal bone cyst (ABC) is an expansile cystic lesion that may affect any bone of the skeleton. Although exceedingly rare, lesions with histomorphologic characteristics of an ABC have reportedly originated within soft tissue. Extraskeletal ABC may mimic a variety of benign and malignant lesions and can be confused with other more common or rare giant cell-rich tumors of soft tissue, especially myositis ossificans. Clinical, radiological and histologic correlation is crucial in reaching the correct diagnosis...
July 6, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28754575/heterotopic-bone-induction-via-bmp-signaling-potential-therapeutic-targets-for-fibrodysplasia-ossificans-progressiva
#16
Takenobu Katagiri, Sho Tsukamoto, Mai Kuratani
More than 50years ago, Marshal M. Urist detected "heterotopic bone-inducing activity" in demineralized bone matrix. This unique activity was referred to as "bone morphogenetic protein (BMP)" because it was sensitive to trypsin digestion. Purification of the bone-inducing activity from demineralized bone matrix using a bone-inducing assay in vivo indicated that the original "BMP" consisted of a mixture of new members of the transforming growth factor-β (TGF-β) family. The establishment of new in vitro assay systems that reflect the bone-inducing activity of BMPs in vivo have revealed the functional receptors and downstream effectors of BMPs...
July 25, 2017: Bone
https://www.readbyqxmd.com/read/28751434/atypical-presentation-and-management-of-fibrodysplasia-ossificans-progressiva
#17
André Grenho, Joana Arcângelo, Augusto Martins
We report a case of an 18-year-old woman, with bilateral acute inflammatory pain on the hip area, during the premenstrual period, and progressive increase in volume and rigidity of both hips. Bilateral exuberant soft tissue calcifications were present on the radiographic exams, and the patient also presented with bilateral short-length hallux valgus. A heterozygous mutation in the protein kinase domain of ACVR1 gene was found, allowing the diagnosis of fibrodysplasia ossificans progressive. Due to the relation between the disease flares and the premenstrual period, the patient was put into a chemically induced amenorrhea, with no new inflammatory crises since...
July 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28745249/-a-hardening-in-the-forearm-after-an-olecranon-fracture
#18
C M M Peeters, J C Sluimer, T Gosens
A 77-year-old male who had had an olecranon fracture 15 years ago presented himself with mild pain of the right elbow. Physical examination revealed painless hardening of the finger and wrist flexors in an area of 10 cm from the origin. Conventional radiographs showed a mature myositis ossificans.
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28741585/a-fatal-case-of-metastatic-squamous-cell-carcinoma-in-a-patient-with-myositis-ossificans-traumatica
#19
I N Vlasveld, H Scheper, J Stalenhoef, J M Baas, J van Dissel
Myositis ossificans traumatica is a rare disease associated with chronic wounds and fistulae. Chronic ulcers, fistulae and wounds can transform into squamous cell carcinoma, the so-called Marjolin's ulcer. We describe a rapid, progressive and fulminant course of a metastatic squamous cell carcinoma arising from a chronic wound in a patient with myositis ossificans traumatica.
July 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28736245/early-clinical-observations-on-the-use-of-imatinib-mesylate-in-fop-a-report-of-seven-cases
#20
Frederick S Kaplan, Jeffrey R Andolina, Peter C Adamson, David T Teachey, Jerry Z Finklestein, David H Ebb, Benjamin Whitehead, Benjamin Jacobs, David M Siegel, Richard Keen, Edward Hsiao, Robert J Pignolo
BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is an ultrarare genetic disorder of progressive, disabling heterotopic ossification (HO) for which there is presently no definitive treatment. Research studies have identified multiple potential targets for therapy in FOP, and novel drug candidates are being developed for testing in clinical trials. A complementary approach seeks to identify approved drugs that could be re-purposed for off-label use against defined targets in FOP...
July 20, 2017: Bone
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