Caseinolytic peptidase B protein homolog

Definition:

Functions as a regulatory ATPase and participates in secretion/protein trafficking process. Involved in mitochondrial-mediated antiviral innate immunity, activates RIG-I-mediated signal transduction and production of IFNB1 and pro-inflammatory cytokine IL6. Has ATP-dependent protein disaggregase activity and is required to maintain the solubility of key mitochondrial proteins. Plays a role in granulocyte differentiation.

References:

[1]. Julia T Warren, et al. Heterozygous variants of CLPB are a cause of severe congenital neutropenia. Blood. 2022 Feb 3;139(5):779-791. [Content Brief]

[2]. Takahiro Yoshinaka, et al. Structural Basis of Mitochondrial Scaffolds by Prohibitin Complexes: Insight into a Role of the Coiled-Coil Region. iScience. 2019 Sep 27;19:1065-1078. [Content Brief]

[3]. Ryan R Cupo, et al. Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations. Elife. 2020 Jun 23;9:e55279. [Content Brief]

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