scholarly journals A Zebrafish Model of Human Barth Syndrome Reveals the Essential Role of Tafazzin in Cardiac Development and Function

2006 ◽  
Vol 99 (2) ◽  
pp. 201-208 ◽  
Author(s):  
Zaza Khuchua ◽  
Zou Yue ◽  
Lorene Batts ◽  
Arnold W. Strauss
PLoS Biology ◽  
2021 ◽  
Vol 19 (11) ◽  
pp. e3001444
Author(s):  
Nina L. Kikel-Coury ◽  
Jacob P. Brandt ◽  
Isabel A. Correia ◽  
Michael R. O’Dea ◽  
Dana F. DeSantis ◽  
...  

Glial cells are essential for functionality of the nervous system. Growing evidence underscores the importance of astrocytes; however, analogous astroglia in peripheral organs are poorly understood. Using confocal time-lapse imaging, fate mapping, and mutant genesis in a zebrafish model, we identify a neural crest–derived glial cell, termed nexus glia, which utilizes Meteorin signaling via Jak/Stat3 to drive differentiation and regulate heart rate and rhythm. Nexus glia are labeled with gfap, glast, and glutamine synthetase, markers that typically denote astroglia cells. Further, analysis of single-cell sequencing datasets of human and murine hearts across ages reveals astrocyte-like cells, which we confirm through a multispecies approach. We show that cardiac nexus glia at the outflow tract are critical regulators of both the sympathetic and parasympathetic system. These data establish the crucial role of glia on cardiac homeostasis and provide a description of nexus glia in the PNS.


1996 ◽  
Vol 93 (23) ◽  
pp. 12974-12979 ◽  
Author(s):  
M. Jaber ◽  
W. J. Koch ◽  
H. Rockman ◽  
B. Smith ◽  
R. A. Bond ◽  
...  

2020 ◽  
Vol 118 (3) ◽  
pp. 258a
Author(s):  
Laszlo Csernoch ◽  
Mónika Gönczi ◽  
Zsolt Ráduly ◽  
László Szabó ◽  
Nóra Dobrosi ◽  
...  

2006 ◽  
Vol 343 (1) ◽  
pp. 144-151 ◽  
Author(s):  
Kiyonori Togi ◽  
Yoshinori Yoshida ◽  
Hironobu Matsumae ◽  
Yasuhiro Nakashima ◽  
Toru Kita ◽  
...  

Author(s):  
Weinberg-Shukron A ◽  
Rachmiel M ◽  
Renbaum P ◽  
Gulsuner S ◽  
Walsh T ◽  
...  

2000 ◽  
Vol 165 (4) ◽  
pp. 1738-1742 ◽  
Author(s):  
Ari Hashimoto ◽  
Kiyoshi Takeda ◽  
Muneo Inaba ◽  
Masayuki Sekimata ◽  
Tsuneyasu Kaisho ◽  
...  

2020 ◽  
Vol 8 (2) ◽  
pp. 10
Author(s):  
Michael T. Chin ◽  
Simon J. Conway

Tafazzin, an enzyme associated with the rare inherited x-linked disorder Barth Syndrome, is a nuclear encoded mitochondrial transacylase that is highly conserved across multiple species and plays an important role in mitochondrial function. Numerous studies have elucidated the mechanisms by which Tafazzin affects mitochondrial function, but its effects on development and susceptibility to adult disease are incompletely understood. The purpose of this review is to highlight previous functional studies across a variety of model organisms, introduce recent studies that show an important role in development, and also to provide an update on the role of Tafazzin in human disease. The profound effects of Tafazzin on cardiac development and adult cardiac homeostasis will be emphasized. These studies underscore the importance of mitochondrial function in cardiac development and disease, and also introduce the concept of Tafazzin as a potential therapeutic modality.


2022 ◽  
pp. 165-178
Author(s):  
Chunlong Mu ◽  
◽  
Weiyun Zhu ◽  

The gut epithelium acts as a barrier to the gut environment. The integrity of the epithelial structure and function is thus critical for microbiome-host interaction. The gut microbiome can regulate the utilization and synthesis of mucin, the expressions of the intercellular junction complex, and the functioning of specific epithelial cells, such as enterochromaffin cells and stem cells in pigs. The factors involved include microbial metabolites, especially short-chain fatty acids and host-microbe co-metabolism. Recent studies have revealed the essential role of amino acid metabolism in regulating the gut microbiome and epithelial barrier. This chapter discusses how the pig gut microbiome modulates epithelial structure and function, highlighting findings that reflect the relationship between the gut microbiome, intestinal structure and function.


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