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Journal: Biochemical Society Transactions
Biochem Soc Trans (2021) 49 (5): 2443–2454.
Published: 28 October 2021
... and HOIP and an adaptor protein SHARPIN. Together they are responsible for M1-linked ubiquitination of substrates primarily related to immune signaling and cell death pathways. Consequently, severe immunodeficiency is a hallmark of many LUBAC deficient patients. Remarkably, all HOIL-1L deficient patients...