2016年9月15日 · Strikingly, the extended SPATA2 fragment formed a trimeric complex with CYLD and HOIP that eluted with an MW of 170 kDa, indicative of a stable 2:2:2 complex (Figure 4G, red, green, and orange curves; see Supplemental Experimental Procedures for details on stoichiometry calculation).

2021年6月18日 · HOIP has a catalytic centre in its RING2 domain responsible for assembly of linear ubiquitin chains, while HOIL-1L and SHARPIN have been recognized as accessory proteins for the process. It is more recent that HOIL-1L has been shown to catalyse ubiquitination (Pao et al., 2018; Stieglitz et al., 2012; Smit et al., 2013; Tatematsu et al., 2008).

2019年3月17日 · Here we describe a second case of HOIP deficiency with compound heterozygous mutations in RNF31, who was clinically diagnosed with common variable immunodeficiency (CVID). Similar to the first case with HOIP deficiency, the patient presented with systemic inflammatory features but without evidence of amylopectinosis or lymphangiectasia.

HOIL-1L and its binding partner, HOIL-1L interacting protein (HOIP), are essential components of linear ubiquitin (Ub) chain assembly complex (LUBAC), a 600-kDa enzyme complex catalyzing elongation of a tandemly connected Ub chain, which serve as a regulator of NF-κB activation.

2023年10月10日 · LUBAC comprised by HOIP, HOIL-1L, and SHARPIN as a sole E3 ligase catalyzes the generation of linear ubiquitin chains, and it is simultaneously adjusted by deubiquitinases such as OTULIN and CYLD.

2014年5月8日 · Crystal structures and nuclear magnetic resonance experiments reveal the molecular basis for the high-affinity interaction and explain why OTULIN binds the HOIP PUB domain specifically. Analysis of LUBAC-induced NFκB signaling suggests that OTULIN needs to be present on LUBAC in order to restrict Met1-polyUb signaling.

2020年12月7日 · OTULIN is an OTU DUB that exclusively hydrolyzes Met1-linked poly-ubiquitination. OTULIN interacts with the LUBAC component HOIP and controls Met1 ubiquitination levels, NF-κB activation and cell...

2017年3月25日 · To investigate the physiological function of LUBAC, we first constructed HOIP-knockout (KO) Jurkat cells, derived from human T lymphocytes, by the CRISPR/Cas9 technique (Supplementary Fig. S1A). The genetic ablation of HOIP (HOIP-KO) synergistically decreased the intracellular HOIL-1L and SHARPIN (Fig. 1 B) as described [7], [9].

2012年7月1日 · LUBAC (linear ubiquitin chain assembly complex) is a ubiquitin ligase complex composed of SHARPIN, HOIL-IL and HOIP that generates linear polyubiquitin chains and regulates the NF-κB pathway, which is pivotal in inflammatory and immune responses.

2019年6月27日 · The NF-κB and interferon antiviral signaling pathways play pivotal roles in inflammatory and innate immune responses. The LUBAC ubiquitin ligase complex, composed of the HOIP, HOIL-1L, and SHARPIN subunits, activates the canonical NF-κB pathway through Met1-linked linear ubiquitination.