E3 ubiquitin-protein ligase Midline-1 (UniProt: O15344; also known as EC: 2.3.2.27, Midin, Putative transcription factor XPRF, RING finger protein 59, RING finger protein Midline-1, RING-type E3 ubiquitin transferase Midline-1, Tripartite motif-containing protein 18) is encoded by the MID1 (also known as FXY, RNF59, TRIM18, XPRF) gene (Gene ID: 4281) in human. Midlin is homodimeric protein with E3 ubiquitin ligase activity towards IGBP1, promoting its monoubiquitination that results in deprotection of the catalytic subunit of protein phosphatase PP2A and its subsequent degradation by polyubiquitination. It can also form heterodimer with MID2. It associates with microtubules throughout the cell cycle, co-localizing with cytoplasmic fibers in interphase and with the mitotic spindle and midbodies during mitosis and cytokinesis. It is highly expressed in fetal kidney, followed by brain and lung. Lower levels are observed in fetal liver. In the adult, it is most abundant in heart, placenta, and brain. Midlin has multiple zinc binding sites and possesses three zinc fingers (one RING type and two B box-type). Mutations in MID1 gene are reported to cause Opitz GBBB syndrome 1 that is characterized by reduced affinity for microtubules, hypertelorism, and genital-urinary defects.
Synonyms: E3 ubiquitin-protein ligase Midline-1, EC: 2.3.2.27, Midin, Putative transcription factor XPRF, RING finger protein 59, RING finger protein Midline-1, RING-type E3 ubiquitin transferase Midline-1, Tripartite motif-containing protein 18
Application: Research CategoryNeuroscience
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