• Brittany Bodnar, Arianna DeGruttola, Yuanjun Zhu, Yuan Lin, Yonggang Zhang, Xianming Mo, and Wenhui Hu.
• Center for Metabolic Disease Research, Temple University Lewis Katz School of Medicine, Philadelphia, Pennsylvania; MD/PhD and Biomedical Sciences Graduate Program, Temple University Lewis Katz School of Medicine, Philadelphia, Pennsylvania.
• Transl Res. 2020 Oct 1; 224: 55-70.

AbstractNFκB signaling and protein trafficking network play important roles in various biological and pathological processes. NIK-and-IKK2-binding protein (NIBP), also known as trafficking protein particle complex 9 (TRAPPC9), is a prototype member of a novel protein family, and has been shown to regulate both NFκB signaling pathway and protein transport/trafficking. NIBP is extensively expressed in the nervous system and plays an important role in regulating neurogenesis and neuronal differentiation. NIBP/TRAPPC9 mutations have been linked to an autosomal recessive intellectual disability syndrome, called NIBP Syndrome, which is characterized by nonsyndromic autosomal recessive intellectual disability along with other symptoms such as obesity, microcephaly, and facial dysmorphia. As more cases of NIBP Syndrome are identified, new light is being shed on the role of NIBP/TRAPPC9 in the central nervous system developments and diseases. NIBP is also involved in the enteric nervous system. This review will highlight the importance of NIBP/TRAPPC9 in central and enteric nervous system diseases, and the established possible mechanisms for developing a potential therapeutic.Copyright © 2020 Elsevier Inc. All rights reserved.

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