Consequences of Trps1 Deficiency on Dentinogenesis PDF Download
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Author: Morgan A. Goss
Publisher:
ISBN:
Category :
Languages : en
Pages : 70
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Book Description
Dentinogenesis is a complex process of dentin formation. It involves differentiation of odontoblasts, the cells that make dentin, and the formation of a unique extracellular matrix, known as dentin. Dentin is ultimately produced through the interaction of collagen fibers, non-collagenous proteins and hydroxyapatite (HA) crystals. Odontoblasts are cranial neural crest (CNC) ectomesenchymal derived cells. Trps1 is a novel transcription factor believed to play an important role in both tooth formation and the mineralization process. TRPS1 gene mutations are known to result in dental and skeletal abnormalities manifested in humans as tricho-rhino-phalangeal syndrome (TRPS) and Ambras syndrome. Trps1 expression varies throughout odontoblast development. Strongly expressed in preodontoblasts, Trps1 then decreases in mature secretory cells at onset of mineralization; thus its downregulation coincides with terminal differentiation of odontoblasts. Our laboratory recently demonstrated that sustained high expression of Trps1 in secretory odontoblasts represses secretion and mineralization of dentin matrix in vitro. However, the specific role Trps1 plays in dentinogenesis remains unclear. To better understand the function of Trps1 in odontoblast maturation, ex vivo tooth organ culture studies were performed. Trps1 knockout mice (KO) are perinatal lethal, therefore dentinogenesis has not been studied in these mice until now. Analyses were performed to define whether Trps1-deficiency affects odontoblast maturation and/or odontoblast function. We found that Trps1 KO mice were able to form differentiated odontoblasts, capable of secreting a predentin matrix. However, mature odontoblasts in Trps1 KO mice were unable to produce matrix vesicles, an important component that supports the mineralization process. Altered expression of several key osteogenic factors (Runx2, Sp7, TNAP) in Trps1 KO tooth organs revealed that Trps1 functions in both a cell autonomous and non-autonomous fashion in different areas of the developing molar. Furthermore, analyses of embryonic day 18.5 Trps1 KO molars revealed Trps1-deficienct mice exhibit a smaller first mandibular molar compared to WT, mimicking a dental phenotype seen in human patients affected with TRPS. Results of this project have provided first insight into the role of Trps1 in odontoblast maturation and allowed for better understanding of the molecular mechanisms of dentin formation.
Author: Morgan A. Goss
Publisher:
ISBN:
Category :
Languages : en
Pages : 70
Get Book Here
Book Description
Dentinogenesis is a complex process of dentin formation. It involves differentiation of odontoblasts, the cells that make dentin, and the formation of a unique extracellular matrix, known as dentin. Dentin is ultimately produced through the interaction of collagen fibers, non-collagenous proteins and hydroxyapatite (HA) crystals. Odontoblasts are cranial neural crest (CNC) ectomesenchymal derived cells. Trps1 is a novel transcription factor believed to play an important role in both tooth formation and the mineralization process. TRPS1 gene mutations are known to result in dental and skeletal abnormalities manifested in humans as tricho-rhino-phalangeal syndrome (TRPS) and Ambras syndrome. Trps1 expression varies throughout odontoblast development. Strongly expressed in preodontoblasts, Trps1 then decreases in mature secretory cells at onset of mineralization; thus its downregulation coincides with terminal differentiation of odontoblasts. Our laboratory recently demonstrated that sustained high expression of Trps1 in secretory odontoblasts represses secretion and mineralization of dentin matrix in vitro. However, the specific role Trps1 plays in dentinogenesis remains unclear. To better understand the function of Trps1 in odontoblast maturation, ex vivo tooth organ culture studies were performed. Trps1 knockout mice (KO) are perinatal lethal, therefore dentinogenesis has not been studied in these mice until now. Analyses were performed to define whether Trps1-deficiency affects odontoblast maturation and/or odontoblast function. We found that Trps1 KO mice were able to form differentiated odontoblasts, capable of secreting a predentin matrix. However, mature odontoblasts in Trps1 KO mice were unable to produce matrix vesicles, an important component that supports the mineralization process. Altered expression of several key osteogenic factors (Runx2, Sp7, TNAP) in Trps1 KO tooth organs revealed that Trps1 functions in both a cell autonomous and non-autonomous fashion in different areas of the developing molar. Furthermore, analyses of embryonic day 18.5 Trps1 KO molars revealed Trps1-deficienct mice exhibit a smaller first mandibular molar compared to WT, mimicking a dental phenotype seen in human patients affected with TRPS. Results of this project have provided first insight into the role of Trps1 in odontoblast maturation and allowed for better understanding of the molecular mechanisms of dentin formation.
Author: United Nations. Scientific Committee on the Effects of Atomic Radiation
Publisher: The Committee
ISBN:
Category : Medical
Languages : en
Pages : 166
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Book Description
The 2001 report completed a comprehensive review of the risks to offspring following parental exposure to radiation. The review included an evaluation of those diseases which have both hereditary and environmental components. The major finding is that the total hereditary risk to the first generation following radiation is less than one tenth of the risk of fatal carcinogenesis following irrradiation. The Committee concluded that a sounder basis now exists for estimating the hereditary risks of radiation exposure. This is due to advances in molecular genetics, and in the evaluation of multifactorial diseases, such as coronary heart disease.
Author: Margarethe Geiger
Publisher: Springer
ISBN: 3319227114
Category : Science
Languages : en
Pages : 333
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Book Description
The book provides an comprehensive overview on biology, genetics and cellular functions of serpins (serine protease inhibitors) in health and disease. With over 1000 members serpins are the most diverse family of protease inhibitors. Latest groundbreaking research findings are presented and broaden the understanding on inhibitory and non-inhibitory serpins, not only in mammalian organisms but also in insects, worms, plants and viruses.
Author: Pauline M. Camacho
Publisher: Springer
ISBN: 3030036944
Category : Medical
Languages : en
Pages : 271
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Book Description
This concise, case-based text discusses the current state of the art for the diagnosis and management of metabolic bones diseases. Each chapter opens with a unique case presentation and utilizes a consistent format that includes relevant anatomy, physiology, and pathophysiology as well as examination, treatment approaches and clinical outcomes. Topics covered include osteoporosis, rickets and osteomalacia, hypoparathyroidism and pseudohypoparathyroidism, osteogenesis imperfecta, Paget's disease of bone, calcium and phosphorous disorders, hypophosphatasia, sclerotic bone disorders, fibrous and osteochondroplasia, and other malignancies of bone. Written and edited by experts in the field, Metabolic Bone Diseases is a valuable resource for practicing endocrinologists, rheumatologists and orthopedic surgeons, residents and fellows.
Author: Maria del Carmen Cardenas-Aguayo
Publisher: BoD – Books on Demand
ISBN: 9535126946
Category : Medical
Languages : en
Pages : 176
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Book Description
Restricted growth conditions are a group of genetic disorders with primary effect on growth (short stature); it is very heterogeneous and comprises two important categories: skeletal dysplasia and different genetic syndromes with primary effect on growth. It could also be caused by a medical condition. The book contains chapters regarding different aspects of the study of restricted growth that are divided into three broad sections. Section I: Defining Restricted Growth, Section II: Genetics and Diagnosis of Restricted Growth, and Section III: Signaling Pathways and Molecular Mechanisms of Restricted Growth. The book presents comprehensive reviews of each topic written by experts in the field. It will be the most valuable tool for physicians and life science researchers and students. We hope that the book will motivate discussion and research in this important health problem, setting the path for better therapeutic approaches.
Author: Jürgen W. Spranger
Publisher: Oxford University Press, USA
ISBN: 0190626658
Category : Medical
Languages : en
Pages : 929
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Book Description
The definitive guide to genetic bone disorders, now revised and expanded with glossy photographs and radiographs "Brilliantly written and produced and deserves to be on the shelves of all pediatric radiologists. It should also be available to geneticists, counselors, and pediatricians." --Radiology This updated and expanded fourth edition of Bone Dysplasias presents age-related radiographs, photographs and clinical guidelines for more than 250 rare constitutional skeletal diseases. Focusing on diagnostically essential imaging and clinical features, each chapter is supplemented with prognostic and therapeutic information, a guide to differential diagnoses, and a short list of the most relevant publications. Organized in accordance with the most recent International Nosology and Classification of Genetic Skeletal Disorders, this new Bone Dysplasias distills the insights of a small, world-class author team on diagnosis and clinical approaches to this most difficult class of disorders.
Author: Manish Kumar Varshney
Publisher: JP Medical Ltd
ISBN: 9352501756
Category : Medical
Languages : en
Pages : 981
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Book Description
Essential Orthopedics: Principles & Practice is an extensive, illustrated guide to the field of orthopaedics. Principles and practice for shoulder, hip, spine, hand, foot and ankle are covered, including anatomy, physiology, pathology and diseases. Essential Orthopedics: Principles & Practice includes all modern research methodologies, such as biostatistics, advanced imaging and gene therapy. Enhanced by 2000 full colour illustrations this is a comprehensive resource for all interns, residents and orthopaedic surgeons.
Author: Philippa H. Francis-West
Publisher: Springer Science & Business Media
ISBN: 3642555705
Category : Science
Languages : en
Pages : 217
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Book Description
Craniofacial development is a multistep and intricate process initially involving a number of inductive interactions that control neural and neural crest development, which are followed by a series of epithelial-mesenchymal interactions that control outgrowth, patterning, and skeletal differentiation. Certain aspects of craniofacial development are unique developmental processes in higher vertebrates. First, in higher vertebrates the cranial neural crest, in contrast to the trunk neural crest, gives rise to the skeletal structures. These skeletal elements include those comprising mem brane bone and secondary cartilage, which with the exception of the clavicle are tissue types found exclusively in the head in higher vertebrates. Second, with the exception of the tongue, the origin of the musculature is distinct from other regions of the body. The body and tongue muscles are formed from the segmented epithelial somites whilst the head musculature is formed from unsegmented paraxial and prechordal mesoderm. Furthermore, the signalling cascades that control myogenic differentia tion appear to be distinct as determined by gene expression and the response of myogenic cells to growth factors. Finally, the neurogenic placodes, which give rise to the sensory organs and some cranial ganglia, are only found in the head. Over recent years, there have been significant advances in our knowledge of the molecular proc esses that control craniofacial development in a number of animal models. This has given insight into the genes that control many aspects of head development from the initial induction of the head to the final stages of differentiation.
Author: Connie M. Weaver
Publisher: Springer Science & Business Media
ISBN: 1592599613
Category : Science
Languages : en
Pages : 442
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Book Description
The Nutrition and Health Series of books have had great success because each volume has the consistent overriding mission of providing health professionals with texts that are essential because each includes (1) a synthesis of the state of the science; (2) timely, in-depth reviews by the leading researchers in their respective fields; (3) extensive, - to-date fully annotated reference lists; (4) a detailed index; (5) relevant tables and figures; (6) identification of paradigm shifts and the consequences; (7) virtually no overlap of information between chapters, but targeted, interchapter referrals; (8) suggestions of areas for future research; and (9) balanced, data-driven answers to patient/health prof- sionals’ questions that are based on the totality of evidence rather than the findings of any single study. The series volumes are not the outcome of a symposium. Rather, each editor has the potential to examine a chosen area with a broad perspective, both in subject matter as well as in the choice of chapter authors. The international perspective, especially with regard to public health initiatives, is emphasized where appropriate. The editors, whose trainings are both research- and practice-oriented, have the opportunity to develop a primary objective for their book; define the scope and focus, and then invite the leading authorities from around the world to be part of their initiative. The authors are encouraged to provide an overview of the field, discuss their own research, and relate the research findings to potential human health consequences.
Author: Roger E. Stevenson
Publisher: Oxford University Press
ISBN: 019974808X
Category : Medical
Languages : en
Pages : 1510
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Book Description
This widely acclaimed reference work gives a comprehensive survey of all significant human malformations and related anomalies from the perspective of the clinician. The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly. When known, the molecular or other pathogenetic basis for the malformation is given. Most anomalies are illustrated by photographs or drawings. Specific malformations are linked to syndromes through the extensive use of differential diagnosis tables. Over a decade has passed since the first edition of this book was published, and the revised edition fully incorporates the advances made in the field during the intervening years.. It reflects new understanding of human developmental biology that has emerged from molecular, cytogenetic, and biochemical studies; new observations by clinicians as well as enhanced diagnostic and prevention capacities; and more accurate and comprehensive epidemiology. By condensing much of the information presented in the first volume of the previous edition, and exercising rigorous editorial control, Drs. Stevenson and Hall and their contributors have managed to update the book while reducing its size to that of a single volume. All clinicians and scientists interested in birth defects, including pediatricians, geneticists, genetic counselors, obstetricians, and pediatric pathologists, will find this book to be an invaluable source of information.
