A new murine model of Barth syndrome neutropenia links TAFAZZIN deficiency to increased ER stress-induced apoptosis

Barth syndrome is an inherited X-linked disorder that leads to cardiomyopathy, skeletal myopathy, and neutropenia. These symptoms result from the loss of function of the enzyme TAFAZZIN, a transacylase located in the inner mitochondrial membrane that is responsible for the final steps of cardiolipin...

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Detalles Bibliográficos
Autores principales: Sohn, Jihee, Milosevic, Jelena, Brouse, Thomas, Aziz, Najihah, Elkhoury, Jenna, Wang, Suya, Hauschild, Alexander, van Gastel, Nick, Cetinbas, Murat, Tufa, Sara F., Keene, Douglas R., Sadreyev, Ruslan I., Pu, William T., Sykes, David B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society of Hematology 2022
Materias:
Phagocytes, Granulocytes, and Myelopoiesis
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9043941/
https://www.ncbi.nlm.nih.gov/pubmed/34979560
http://dx.doi.org/10.1182/bloodadvances.2021005720

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9043941/
https://www.ncbi.nlm.nih.gov/pubmed/34979560
http://dx.doi.org/10.1182/bloodadvances.2021005720

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