Loss of Fic causes progressive neurodegeneration in a Drosophila model of hereditary spastic paraplegia

Lobato, Amanda G.; Ortiz-Vega, Natalie; Canic, Tijana; Tao, Xianzun; Bucan, Nika; Ruan, Kai; Rebelo, Adriana P.; Schule, Rebecca; Zuchner, Stephan; Syed, Sheyum; Zhai, R. Grace

doi:10.1016/j.bbadis.2024.167348

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Diseas

2024

DOI: 10.1016/j.bbadis.2024.167348

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Loss of Fic causes progressive neurodegeneration in a Drosophila model of hereditary spastic paraplegia

Amanda G. Lobato,

Natalie Ortiz-Vega,

Tijana Canic

et al.

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Dysregulation of FicD AMPylation causes diabetes by disrupting pancreatic endocrine homeostasis

Casey,

Stewart,

Zaidi

et al. 2024

Preprint

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Bi-functional enzyme FicD regulates the endoplasmic reticulum chaperone BiP using AMPylation and deAMPylation during ER homeostasis and stress, respectively. Human FicD with an arginine-to-serine mutation disrupts FicD deAMPylation activity resulting in severe neonatal diabetes. We generated the FicDR371S mutation in mice to create a pre-clinical murine model for neonatal diabetes. We observed elevated BiP AMPylation levels across multiple tissues and signature markers for diabetes including glucose intolerance and reduced serum insulin levels. While the pancreas of FicDR371S mice appeared normal at birth, adult FicDR371S mice displayed disturbed pancreatic islet organization that progressed with age. FicDR371S mice provide a preclinical mouse model for the study of UPR associated diabetes and demonstrate the essentiality of FicD for tissue resilience.

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Dysregulation of FicD AMPylation causes diabetes by disrupting pancreatic endocrine homeostasis

Casey,

Stewart,

Zaidi

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

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Loss of Fic causes progressive neurodegeneration in a Drosophila model of hereditary spastic paraplegia

Cited by 1 publication

References 77 publications

Dysregulation of FicD AMPylation causes diabetes by disrupting pancreatic endocrine homeostasis

Dysregulation of FicD AMPylation causes diabetes by disrupting pancreatic endocrine homeostasis

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