FZR1
Substrate-specific adapter for the anaphase promoting complex/cyclosome (APC/C) E3 ubiquitin-protein ligase complex. Associates with the APC/C in late mitosis, in replacement of CDC20, and activates the APC/C during anaphase and telophase. The APC/C remains active in degrading substrates to ensure that positive regulators of the cell cycle do not accumulate prematurely. At the G1/S transition FZR1 is phosphorylated, leading to its dissociation from the APC/C. Following DNA damage, it is required for the G2 DNA damage checkpoint: its dephosphorylation and reassociation with the APC/C leads to the ubiquitination of PLK1, preventing entry into mitosis. Acts as an adapter for APC/C to target the DNA-end resection factor RBBP8/CtIP for ubiquitination and subsequent proteasomal degradation. Through the regulation of RBBP8/CtIP protein turnover, may play a role in DNA damage response, favoring DNA double-strand repair through error-prone non-homologous end joining (NHEJ) over error-free, RBBP8-mediated homologous recombination (HR). Belongs to the WD repeat CDC20/Fizzy family. Isoform 2 is expressed at high levels in heart, liver, spleen and some cancer cell lines whereas isoform 3 is expressed only at low levels in these tissues. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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Protein type: Ubiquitin conjugating system |
Chromosomal Location of human Ortholog: 19p13.3 |
Cellular Component:
anaphase-promoting complex; cytosol; nuclear membrane; nucleoplasm; nucleus
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Molecular Function:
anaphase-promoting complex binding; protein binding; ubiquitin ligase activator activity; ubiquitin ligase-substrate adaptor activity
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Biological Process:
anaphase-promoting complex-dependent catabolic process; cell division; DNA repair; lens fiber cell differentiation; mitotic G2 DNA damage checkpoint signaling; negative regulation of cellular senescence; positive regulation of anaphase-promoting complex-dependent catabolic process; positive regulation of cell population proliferation; positive regulation of ubiquitin protein ligase activity; protein K11-linked ubiquitination; regulation of meiotic cell cycle; regulation of meiotic nuclear division; regulation of mitotic cell cycle
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Disease: Developmental And Epileptic Encephalopathy 109
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Reference #:
Q9UM11
(UniProtKB)
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Alt. Names/Synonyms: CDC20 homolog 1; CDC20-like 1b; CDC20-like protein 1; CDC20C; CDH1; Cdh1/Hct1 homolog; fizzy and cell division cycle 20 related 1; Fizzy-related protein homolog; fizzy/cell division cycle 20 related 1; fizzy/cell division cycle 20 related 1 (Drosophila); FYR; FZR; FZR1; FZR2; HCDH; hCDH1; KIAA1242
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Gene Symbols: FZR1
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Molecular weight:
55,179 Da
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Basal Isoelectric point:
9.41
Predict pI for various phosphorylation states
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