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Correction to: FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death.

ABSTRACT: Pulmonary fibrosis is a severely disabling disease often leading to death. CCN2 (Cellular Communication Network factor 2, also known as CTGF) is a known mediator of fibrosis and clinical trials studying anti-CCN2 efficacy in pulmonary fibrosis are currently underway. Fork head box D1 (FoxD1) transcription factor is transiently expressed in several mesenchymal cell types, including those of fetal lungs. Differentiation of FoxD1-progenitor derived pericytes into myofibroblasts involves CCN2 expression and contributes importantly to maladaptive tissue remodeling in for example kidney and lung fibrosis models. To generate a model for studying the contribution of CCN2 expression in FoxD1-progenitor derived cells to development of fibrotic tissue remodeling, we set out to establish a FoxD1Cre - CCN2flox/flox mouse colony. However, all double-transgenic mice died soon after birth due to asphyxia. Histopathological examination revealed a reduction in alveolar space and lung weight, and subtle axial (thoracic and cervical) skeletal deformities. Together with the previously reported association of a FoxD1 containing locus with human adolescent idiopathic scoliosis, our data suggest that the fatal pulmonary hypoplasia resulting from selective deletion of CCN2 from FoxD1-progenitor derived mesenchymal cells developed secondary to impaired breathing movements due to aberrant axial skeletogenesis.

SUBMITTER: Falke LL

PROVIDER: S-EPMC7176786 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature

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Correction to: FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death.

Falke Lucas L LL He Nannan N Chuva de Sousa Lopes Susana M SM Broekhuizen Roel R Lyons Karen K Nguyen Tri Q TQ Goldschmeding Roel R

Journal of cell communication and signaling 20200301 1

Pulmonary fibrosis is a severely disabling disease often leading to death. CCN2 (Cellular Communication Network factor 2, also known as CTGF) is a known mediator of fibrosis and clinical trials studying anti-CCN2 efficacy in pulmonary fibrosis are currently underway. Fork head box D1 (FoxD1) transcription factor is transiently expressed in several mesenchymal cell types, including those of fetal lungs. Differentiation of FoxD1-progenitor derived pericytes into myofibroblasts involves CCN2 expres ...[more]

PMID: 32291573

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Project description:IntroductionDeformities of the lower extremities can be congenital or acquired. Various surgical treatments have been employed for such disorders including osteotomy followed by either external fixation, internal fixation or external fixator assisted internal fixation. The aim of surgery is correction of deformity and restoration of mechanical axis and joint line. External fixator assisted internal fixation with intramedullary (IM) nail insertion is considered the gold standard, however, it is less commonly practiced as expertise required are usually not available at most centers. This study was conducted to assess the radiological and functional outcomes after fixator assisted IM nailing for correction of lower limbs deformity.MethodsIt was a retrospective study at a tertiary care hospital. All cases of lower limb deformity whose correction was done with fixator assisted IM nailing from 2010 till 2017 were analyzed. Pre Op x-rays and post op x rays were analyzed for Mechanical Axis Deviation (MAD), anatomical Lateral Distal Femoral Angle (aLDFA), mechanical Lateral Distal Femoral Angle (mLDFA) and Medial Proximal Tibial Angle (MPTA), post-operative activity and functional status of the patients. Data was analyzed using SPSS.ResultsThirteen patients were included in the study. Fixator assisted IM nailing was performed on 29 long bones of these patients including 16 femur and 13 tibial deformities. Pre Op and Post Op comparison was done for MAD, aLDFA, mLDFA, MPTA. Pre op mean MAD was 38.87 ± 25.58 post op mean MAD 17.54 ± 12.25 mm. Only 2 of our patients developed knee stiffness for which manipulation under anesthesia was done. One of our patients developed weakness in toe extension, which recovered after 6 months. On follow up evaluation patients had normal range of motion and no functional limitation.ConclusionFixator assisted IM nailing for deformity correction is a better option, because it has advantages of both external fixator and internal fixator. Knee stiffness associated with external fixator can be prevented. It is more convenient for patient.

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Correction to: FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death.

Publications

Correction to: FoxD1-driven CCN2 deletion causes axial skeletal deformities, pulmonary hypoplasia, and neonatal asphyctic death.

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