Multi-omics characterization of 2 patients with biallelic HYOU1 variants reveals that HYOU1 deficiency impairs the unfolded protein response, disrupts early B cell development, and alters neutrophil granulation as well as IFN signalling, further establishing ER proteostasis as a contributor to human immune homeostasis.
Aida Idani, S. Bibi-Triki, Tristan Stemmelen et al.· Journal of human immunity· 0 citations
The results define cis-PTase as a master regulator of CNS development and function and establish that its monoallelic debilitating variants cause a novel congenital disorder of glycosylation associated with aberrant levels of neuronal proteins and lipids.
Afitz Da Silva, Merrick S. Fallah, Samuel Boris Tene Tadoum et al.· Brain : a journal of neurolo...· 0 citations
Histopathological, electron microscopical, biochemical, and molecular characterization of muscle and heart tissue indicated mitochondrial dysfunction with combined complex deficiency associated with a mitochondrial DNA maintenance disorder primarily expressed in the heart.
Rodrigo T. Starosta, Marisa W. Friederich, Graeme Preston et al.· American Journal of Medical...· 1 citation
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