D
IPR032714

Cilium assembly protein DZIP1, N-terminal

InterPro entry
Short nameDZIP1_N

Description

This entry represents the N-terminal region of the cilium assembly protein DZIP1, which acts as a molecular adapter that recruits protein complexes required for cilium assembly and function to the cilium basal body
[2, 5, 3, 4]
. It also recruits the BBSome, a complex involved in cilium biogenesis, by bridging it to PCM1 at the centriolar satellites of the cilium
[3]
. DZIP1 is involved in spermatogenesis through its role in organization of the basal body and assembly of the sperm flagellum
[4]
and it is also indirectly involved in heart development through its function in ciliogenesis
[6]
. Zebrafish Dzip1 acts as a permissive factor that is required for the proper regulation of Hedgehog (Hh) target genes in response to Hh signals
[1]
. It is required for establishment of left-right asymmetry during embryogenesis and has a role in primary cilium formation
[2]
.

References

1.The zebrafish iguana locus encodes Dzip1, a novel zinc-finger protein required for proper regulation of Hedgehog signaling. Sekimizu K, Nishioka N, Sasaki H, Takeda H, Karlstrom RO, Kawakami A. Development 131, 2521-32, (2004). View articlePMID: 15115751

2.The Zn finger protein Iguana impacts Hedgehog signaling by promoting ciliogenesis. Glazer AM, Wilkinson AW, Backer CB, Lapan SW, Gutzman JH, Cheeseman IM, Reddien PW. Dev. Biol. 337, 148-56, (2010). View articlePMID: 19852954

3.DAZ-interacting Protein 1 (Dzip1) Phosphorylation by Polo-like Kinase 1 (Plk1) Regulates the Centriolar Satellite Localization of the BBSome Protein during the Cell Cycle. Zhang B, Wang G, Xu X, Yang S, Zhuang T, Wang G, Ren H, Cheng SY, Jiang Q, Zhang C. J Biol Chem 292, 1351-1360, (2017). PMID: 27979967

4.Homozygous mutations in <i>DZIP1</i> can induce asthenoteratospermia with severe MMAF. Lv M, Liu W, Chi W, Ni X, Wang J, Cheng H, Li WY, Yang S, Wu H, Zhang J, Gao Y, Liu C, Li C, Yang C, Tan Q, Tang D, Zhang J, Song B, Chen YJ, Li Q, Zhong Y, Zhang Z, Saiyin H, Jin L, Xu Y, Zhou P, Wei Z, Zhang C, He X, Zhang F, Cao Y. J Med Genet 57, 445-453, (2020). PMID: 32051257

5.Centrosomal protein DZIP1 regulates Hedgehog signaling by promoting cytoplasmic retention of transcription factor GLI3 and affecting ciliogenesis. Wang C, Low WC, Liu A, Wang B. J Biol Chem 288, 29518-29, (2013). PMID: 23955340

6.Primary cilia defects causing mitral valve prolapse. Toomer KA, Yu M, Fulmer D, Guo L, Moore KS, Moore R, Drayton KD, Glover J, Peterson N, Ramos-Ortiz S, Drohan A, Catching BJ, Stairley R, Wessels A, Lipschutz JH, Delling FN, Jeunemaitre X, Dina C, Collins RL, Brand H, Talkowski ME, Del Monte F, Mukherjee R, Awgulewitsch A, Body S, Hardiman G, Hazard ES, da Silveira WA, Wang B, Leyne M, Durst R, Markwald RR, Le Scouarnec S, Hagege A, Le Tourneau T, Kohl P, Rog-Zielinska EA, Ellinor PT, Levine RA, Milan DJ, Schott JJ, Bouatia-Naji N, Slaugenhaupt SA, Norris RA. Sci Transl Med 11, (2019). PMID: 31118289

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