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Koshiba-Takeuchi, K., Takeuchi, J. K., Matsumoto, K., Momose, T., Uno, K., Hoepker, V., Ogura, K., Takahashi, N., Nakamura, H., Yasuda, K., Ogura, T. In a 10-year-old girl with isolated bilateral dorsalization of her fifth fingers and slightly deep fourth web spaces, Al-Qattan et al. (2020) identified a de novo heterozygous 2-basepair duplication in the TBX3 gene ( 601621.0008), resulting in frameshift and premature termination of the protein. The authors suggested that these clinical findings should be considered a forme fruste phenotype of ulnar-mammary syndrome.

In 3 members of a family with ulnar-mammary syndrome, Meneghini et al. (2006) identified a single-basepair insertion ( 601621.0007) in exon 6 of the TBX3 gene, resulting in a frameshift and premature stop codon. This mutation was downstream of the T-box DNA-binding domain and thus did not disrupt or alter the T-domain. The authors reviewed the data on previously reported variants and hypothesized a genotype-phenotype correlation, with mutations that disrupt the T-box DNA-binding domain associated with a more severe phenotype. By microdissection of the mouse ventricular conduction system, followed by serial analysis of gene expression (SAGE) of the left bundle branch, Moskowitz et al. (2007) identified Id2 ( 600386) as a conduction system-specific transcript. Analysis of the Id2 promoter showed that conduction system-specific expression of Id2 was dependent on Nkx2.5 and Tbx5. Moskowitz et al. (2007) concluded that a molecular pathway including Id2, Nkx2.5, and Tbx5 coordinates specification of ventricular myocytes into the ventricular conduction system lineage. Terrett, J. A., Newbury-Ecob, R., Cross, G. S., Fenton, I., Raeburn, J. A., Young, I. D., Brook, J. D. Zhen Yang 1† Qiuning Zhang 2,3† Hongtao Luo 2 Lihua Shao 3 Ruifeng Liu 2 Yarong Kong 2 Xueshan Zhao 4 Yichao Geng 4 Chengcheng Li 4 Xiaohu Wang 1,2,3,4* Nadadur, R. D., Broman, M. T., Boukens, B., Mazurek, S. R., Yang, X., van den Boogaard, M., Bekeny, J., Gadek, M., Ward, T., Zhang, M., Qiao, Y., Martin, J. F., Seidman, C. E., Seidman, J., Christoffels, V., Efimov, I. R., McNally, E. M., Weber, C. R., Moskowitz, I. P.Bruneau, B. G., Nemer, G., Schmitt, J. P., Charron, F., Robitaille, L., Caron, S., Conner, D. A., Gessler, M., Nemer, M., Seidman, C. E., Seidman, J. G. Objective: To analyze the effect of carbon ion ( 12C 6+) radiation may induce bystander effect on A549 cell metastasis and metabonomics. Minn Kota Riptide Ulterra Advanced iPilot Trolling Motor for Saltwater has been redesigned with improved features and Bluetooth connectivity. Complete with iPilot remote, the Riptide Ulterra allows you to Stow, deploy, Trim and operate your motor from anywhere on the Boat.

Parking will be provided free to all outpatients who attend hospital for an appointment at least 3 times within a month and for an overall period of at least 3 months. A ‘month’ is defined as a period of 30 days.

ORIGINAL RESEARCH article

Using lineage tracing in mice, Wang et al. (2015) found that Axin2 ( 604025) identifies a population of proliferating and self-renewing cells adjacent to the central vein in the liver lobule. These pericentral cells express the early liver progenitor marker Tbx3 and are diploid, and thereby differ from mature hepatocytes, which are mostly polyploid. The descendants of pericentral cells differentiate into Tbx3-negative, polyploid hepatocytes, and can replace all hepatocytes along the liver lobule during homeostatic renewal. Adjacent central vein endothelial cells provide Wnt signals that maintain the pericentral cells, thereby constituting the niche. Wang et al. (2015) concluded that they identified a cell population in the liver that subserves homeostatic hepatocyte renewal, characterizes its anatomic niche, and identifies molecular signals that regulate its activity.

Hiroi et al. (2001) found that TBX5 associates with NKX2-5 ( 600584) and synergistically promotes cardiomyocyte differentiation. Both directly bind to the promoter of the gene encoding cardiac-specific natriuretic peptide precursor type A (NPPA; 108780) in tandem, and the 2 transcription factors show synergistic activation. P19CL6 cells efficiently differentiate into beating cardiomyocytes expressing cardiac-specific genes after treatment with 1% dimethyl sulfoxide (DMSO). Hiroi et al. (2001) found that P19CL6 cell lines overexpressing wildtype Tbx5 started to beat earlier and expressed cardiac-specific genes more abundantly than did parental P19CL6 cells, whereas cell lines expressing the G80R mutation ( 601620.0004), which causes substantial cardiac defects with minor skeletal abnormalities in HOS, did not differentiate into beating cardiomyocytes. Contrariwise, the R237Q mutation ( 601620.0003), which causes upper limb malformations without cardiac abnormalities, activated the Nppa promoter to an extent similar to that of wildtype TBX5. In a Czech mother and 2 daughters who were diagnosed with Holt-Oram syndrome, Borozdin et al. (2006) identified a 2.19 to 2.27-Mb contiguous deletion encompassing the TBX5 and TBX3 genes. Clinical reexamination confirmed the presence of features of ulnar-mammary syndrome that were previously unrecognized. Borozdin et al. (2006) noted that the contiguous deletion also included the RBM19 gene ( 616444), but commented that it was unlikely to contribute to or modify the phenotype since all the anomalies present in the affected individuals could be explained by either TBX5 or TBX3 haploinsufficiency.Using lineage tracing in mice, Wang et al. (2015) found that Axin2 (604025) identifies a population of proliferating and self-renewing cells adjacent to the central vein in the liver lobule. These pericentral cells express the early liver progenitor marker Tbx3 and are diploid, and thereby differ from mature hepatocytes, which are mostly polyploid. The descendants of pericentral cells differentiate into Tbx3-negative, polyploid hepatocytes, and can replace all hepatocytes along the liver lobule during homeostatic renewal. Adjacent central vein endothelial cells provide Wnt signals that maintain the pericentral cells, thereby constituting the niche. Wang et al. (2015) concluded that they identified a cell population in the liver that subserves homeostatic hepatocyte renewal, characterizes its anatomic niche, and identifies molecular signals that regulate its activity. The definition of terminal illness is an illness or condition which cannot be cured and is likely to lead to the death of a patient. Palliative Care is a patient requiring support at end of life.