Intersections of Fibrodysplasia Ossificans Progressiva and Traumatic Heterotopic Ossification

被引:9
|
作者
Juan, Conan [1 ]
Bancroft, Alec C. [1 ,2 ]
Choi, Ji Hae [1 ]
Nunez, Johanna H. [1 ]
Pagani, Chase A. [1 ]
Lin, Yen-Sheng [3 ]
Hsiao, Edward C. [4 ,5 ]
Levi, Benjamin [1 ]
机构
[1] Univ Texas Southwestern Med Ctr, Ctr Organogenesis Regenerat & Trauma, Dept Surg, Dallas, TX 75390 USA
[2] Baylor Coll Med, Houston, TX 77030 USA
[3] Univ Texas Southwestern Med Ctr, Dept Orthopaed Surg, Dallas, TX 75390 USA
[4] Univ Calif San Francisco, Inst Human Genet, Dept Med, Div Endocrinol & Metab,Med Ctr, San Francisco, CA 94143 USA
[5] Univ Calif San Francisco, Program Craniofacial Biol, Med Ctr, San Francisco, CA 94143 USA
关键词
heterotopic ossification; fibrodysplasia ossificans progressiva; ectopic bone; ACVR2; ALK2; trauma; MESENCHYMAL STEM-CELLS; ECTOPIC BONE-FORMATION; TGF-BETA; OSTEOGENIC DIFFERENTIATION; TRANSCRIPTION FACTOR; MAMMALIAN TARGET; OSTEOBLASTIC DIFFERENTIATION; OSTEOCALCIN EXPRESSION; STEM/PROGENITOR CELLS; ALKALINE-PHOSPHATASE;
D O I
10.3390/biom14030349
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Heterotopic ossification (HO) is a debilitating pathology where ectopic bone develops in areas of soft tissue. HO can develop as a consequence of traumatic insult or as a result of dysregulated osteogenic signaling, as in the case of the orphan disease fibrodysplasia ossificans progressiva (FOP). Traumatic HO (tHO) formation is mediated by the complex interplay of signaling between progenitor, inflammatory, and nerve cells, among others, making it a challenging process to understand. Research into the pathogenesis of genetically mediated HO (gHO) in FOP has established a pathway involving uninhibited activin-like kinase 2 receptor (ALK2) signaling that leads to downstream osteogenesis. Current methods of diagnosis and treatment lag behind pre-mature HO detection and progressive HO accumulation, resulting in irreversible decreases in range of motion and chronic pain for patients. As such, it is necessary to draw on advancements made in the study of tHO and gHO to better diagnose, comprehend, prevent, and treat both.
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页数:27
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