scholarly journals A phenotypic rescue approach identifies lineage regionalization defects in a mouse model of DiGeorge syndrome

2021 ◽  
Author(s):  
Gabriella Lania ◽  
Monica Franzese ◽  
Adachi Noritaka ◽  
Marchesa Bilio ◽  
Annalaura Russo ◽  
...  

ABSTRACTTBX1 is a key regulator of pharyngeal apparatus (PhAp) development. Vitamin B12 treatment partially rescues aortic arch patterning defects of Tbx1+/- embryos. Here we show that it also improves cardiac outflow tract septation and branchiomeric muscle anomalies of Tbx1 hypomorphic mutants. At molecular level, the in vivo vB12 treatment let us to identify genes that were dysregulated by Tbx1 haploinsufficiency and rescued by treatment. We found that SLUG, encoded by the rescued gene Snai2, identified a population of mesodermal cells that was partially overlapping with but distinct from ISL1+ and TBX1+ populations. In addition, SLUG+ cells were mislocalized and had a greater tendency to aggregate in Tbx1+/- and Tbx1-/- embryos and vB12 treatment restore cellular distribution. Adjacent neural crest-derived mesenchymal cells, which do not express TBX1, were also affected, showing enhanced segregation from cardiopharyngeal mesodermal cells. We propose that TBX1 regulates cell distribution in core mesoderm and the arrangement of multiple lineages within the PhAp.

2017 ◽  
Vol 26 (22) ◽  
pp. 4540-4540
Author(s):  
Gabriella Lania ◽  
Alberto Bresciani ◽  
Monica Bisbocci ◽  
Alessandra Francone ◽  
Vincenza Colonna ◽  
...  

2016 ◽  
pp. ddw267
Author(s):  
Gabriella Lania ◽  
Alberto Bresciani ◽  
Monica Bisbocci ◽  
Alessandra Francone ◽  
Vincenza Colonna ◽  
...  

2017 ◽  
Vol 5 (8) ◽  
pp. 1531-1536 ◽  
Author(s):  
Chenxi Wu ◽  
Hongmei Chen ◽  
Xuan Wu ◽  
Xiuxiu Cong ◽  
Li Wang ◽  
...  

Tumor-induced immune dysfunction has been observed in tumor bearing mice. The nanoparticle cellular distribution is significantly affected by the immune dysfunction, compared with tumor free mice.


2010 ◽  
Vol 80 (45) ◽  
pp. 330-335 ◽  
Author(s):  
Lindsay Helen Allen

Vitamin B12 deficiency is common in people of all ages who consume a low intake of animal-source foods, including populations in developing countries. It is also prevalent among the elderly, even in wealthier countries, due to their malabsorption of B12 from food. Several methods have been applied to diagnose vitamin B12 malabsorption, including Schilling’s test, which is now used rarely, but these do not quantify percent bioavailability. Most of the information on B12 bioavailability from foods was collected 40 to 50 years ago, using radioactive isotopes of cobalt to label the corrinoid ring. The data are sparse, and the level of radioactivity required for in vivo labeling of animal tissues can be prohibitive. A newer method under development uses a low dose of radioactivity as 14C-labeled B12, with measurement of the isotope excreted in urine and feces by accelerator mass spectrometry. This test has revealed that the unabsorbed vitamin is degraded in the intestine. The percent bioavailability is inversely proportional to the dose consumed due to saturation of the active absorption process, even within the range of usual intake from foods. This has important implications for the assessment and interpretation of bioavailability values, setting dietary requirements, and interpreting relationships between intake and status of the vitamin.


2015 ◽  
Vol 63 (S 01) ◽  
Author(s):  
C. Heim ◽  
S. Müller ◽  
B. Weigmann ◽  
M. Ramsperger-Gleixner ◽  
N. Koch ◽  
...  

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