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More evidence that LUBAC has functions beyond immune signalling. Our recent study shows that LUBAC and OTULIN regulate metabolic signalling: specifically they govern AMPK signalling to control metabolic adaption, autophagy, and cell death during energetic stress. This suggests that the metabolic symptoms observed in patients with LUBAC deficiency and ORAS, including glycogen storage disease, lipodystrophy, and liver disease, may be caused directly my metabolic dysregulation rather than being secondary to the systemic inflammation.
nature.com/articles/s4141…
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