CYLDCylindromatosis, Familial
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2016) CYLD limits Lys63- and Met1-linked ubiquitin at receptor complexes to regulate innate immune signaling.
2015) LUBAC-recruited CYLD and A20 regulate gene activation and cell death by exerting opposing effects on linear ubiquitin in signaling complexes.
miR-296-5p Repression of viral EV71 VP3 and 37 replication VP1-coding region miR-23b Repression of viral EV71 3' UTR 38 replication miR373 and Repression of viral EV71 5' UTR 39 miR542-5p replication miRNA-548 Enhancement of IFN-[lambda]1 41 replication of EV71 and VSV miR-146a Enhancement of viral IRAK1 and TRAF6 42 pathogenesis miR-526a Repression of viral CYLD 43 replication miR-146a and Enhancement of EV71- SOS1and 44 miR-370 induced cell apoptosis GADD45[beta] miR-141 Enhancement of EV eIF4E 45 propagation miR-27a Repression of viral EGFR 46 replication miR-1246 Contribution to the DLG3 47 neurological pathogenesis Table 2--MiRNAs involved in CVB3infection.
When CYLD is faulty the response overheats, triggering the formation of tumours.
The problems occur because the damaged CYLD gene can no longer keep in check a molecule called NF-kappaB, important for turning on the inflammatory response.
15 QTL Nearest gene (6) Name Distance (bp) Weaning weight 1 GRIA1 40,884 2 PLCH2 within 3 RAB6A within 4 MSGN1 11,224 5 NUDT18 within Yearling weight 1 LOC100141099 162,878 2 NEBL within 3 MGC140224 37,170 4 LOC520939 51,772 5 ZNF226 142 Carcass weight 1 LOC100139888 260,980 2 TOX within 3 LOC509345 443,377 4 CYLD within 5 DLC1 61,060 Backfat thickness 1 LOC100139637 63,038 2 bta-mir-30a 406,847 3 ETS1 459,952 4 CD9 2,769 5 LMCD1 134,899 Longissimus dorsi muscle area 1 LOC784783 909,550 2 TOX within 3 ST6GALNAC5 within 4 LOC100139888 260,980 5 MIR34A 33 Marbling score 1 LOC100139888 260,980 2 SEMA5A 53426 3 BTBD1 within 4 COBL 109,664 5 LOC509345 443,377 QTL, quantitative trait loci; SNP, single nucleotide polymorphism; MAF, minor allele frequency.
Multiple (familial) trichoepitheliomas: a clinicopathologic and molecular biological study, including CYLD and PTCH gene analysis, of a series of 16 patients.
Hereditary Tumor Syndromes Associated With Cutaneous Nodules Syndrome Mode of Genetic Defect Inheritance Muir-Torre syndrome Autosomal Defective mismatch repair dominant genes hMLH1 and hMSH2 in 70% Gardner syndrome Autosomal APC gene mutations (5q21) (phenotypic variant of dominant FAP) Cowden syndrome (multiple Autosomal PTEN gene mutations hamartoma syndrome) dominant (10q23) Multiple trichoepitheliomas Autosomal CYLD gene mutations (possible phenotypic dominant (16q12-13) variant of Brooke-Spiegler syndrome) Basal cell nevus syndrome Autosomal PTCH gene mutations (Goltz syndrome) dominant (9q22.
283-286) Loss of heterozygosity at 16q12-13 was also described in membranous basal cell adenomas of the parotid gland, (287) and CYLD mutations were found in multiple familial trichoepitheliomas, suggesting that it represents a related condition.
294,295) It appears that CYLD interacts with NF-[kappa]B essential modulator and TNF receptor-associated factor 2, a regulator of I[kappa]B kinase, and is capable of deubiquitinylation through its C-terminal hydrolase domain, leading to subsequent NF-[kappa]B inhibition.
110) The involved gene in all of these syndromes is the CYLD tumor suppressor gene located on band 16q12-13.
131) Conversely, deletion of CYLD, a deubiquitinating enzyme that functions to down-regulate NF-[kappa]B, resulted in more severe colonic inflammation and increased incidence of colonic adenocarcinomas.