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A new spontaneous model of fibrodysplasia ossificans progressiva.
Rothschild, Bruce M. ; Martin, Larry D. ; Timm, Robert M.
Rothschild, Bruce M.
Martin, Larry D.
Timm, Robert M.
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Abstract
Fibrodisplasia ossificante progressiva (FOP) é uma doença genética caracterizada por uma acentuada, progressiva e aparentemente incontrolável ossificação dos tendões, ligamentos, faciais e músculos estriados da formação de osso heterotópico resultando na imobilização em cadeiraa de rodas por 30 anos. Estudos mais avaçados relacionados a sua gestão foi sempre comprometida pela falta de um modelo animal natural. Esqueletos de mamíferos “Defleshed” foram examinados para demonstrar a evidência de formação de osso heterotópico. O cervo-rato do sudeste asiático do gênero Tragulus foi diagnosticado por possuir uma bainha ósseas cobrindo a parte inferior das costas e na região da coxa consistente com a definição clínica da FOP. Esta deposição óssea heterotophica está presente em todos os machos adultos, incluindo os obtidos selvagens e zoológico de animais desta linhagem. Nós relatamos o primeiro exemplo conhecido de natural de fibrodisplasia ossificante progressiva (FOP) em um mamífero não-humano. Tragulus pode oferecer a oportunidade de examinar muitos dos atributos mais importantes da doença experimentalmente
Fibrodysplasia ossificans progressiva (FOP) is a genetic disorder characterized by relentlessly progressive and seemingly uncontrollable progressive ossification of tendons, ligaments, fascia, and striated muscle with heterotopic bone formation resulting in immobilization and wheel chair confinement by age 30. Progress in its management has been compromised by lack of a natural animal model. Defleshed mammal skeletons were examined for evidence of heterotopic bone formation. The Southeast Asian mouse deer of the genus Tragulus was found to have an osseous sheath covering the lower back and upper thigh region consistent with the clinical definition of FOP. This heterotophic bone deposition is present in all adults males, including both wild obtained and zoo bred animals. We report the first known example of spontaneous, naturally occurring fibrodysplasia ossificans progressiva (FOP) in a non-human mammal. Tragulus may offer the opportunity to examine many of the disease’s most significant attributes experimentally.
Fibrodysplasia ossificans progressiva (FOP) is a genetic disorder characterized by relentlessly progressive and seemingly uncontrollable progressive ossification of tendons, ligaments, fascia, and striated muscle with heterotopic bone formation resulting in immobilization and wheel chair confinement by age 30. Progress in its management has been compromised by lack of a natural animal model. Defleshed mammal skeletons were examined for evidence of heterotopic bone formation. The Southeast Asian mouse deer of the genus Tragulus was found to have an osseous sheath covering the lower back and upper thigh region consistent with the clinical definition of FOP. This heterotophic bone deposition is present in all adults males, including both wild obtained and zoo bred animals. We report the first known example of spontaneous, naturally occurring fibrodysplasia ossificans progressiva (FOP) in a non-human mammal. Tragulus may offer the opportunity to examine many of the disease’s most significant attributes experimentally.
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Date
2010-12
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Brazilian Geographical Journal: Geosciences and Humanities Research Medium
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Keywords
animal model, chevrotain, fibrodysplasia ossificans, genetic disorder, heterotopic ossification, mouse deer, ossificação heterotópica, Tragulidae, Tragulus
Citation
Rothschild, B. M., L. D. Martin, and R. M. Timm. 2010. A new spontaneous model of fibrodysplasia ossificans progressiva. Brazilian Geographical Journal: Geosciences and Humanities Research Medium 1(2):228–237.