产品详情 |
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Antigenic Specificity | Cylindromatosis (Turban Tumor Syndrome) (CYLD) (aa 56-70) |
Clone | polyclonal |
Host Species | Rabbit |
Reactive Species | human |
Isotype | n/a |
Format | unconjugated |
Size | 50 µg |
Concentration | n/a |
Applications | Immunocytochemistry (ICC),Western Blotting (WB) |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
Description | Cylindromas are benign adnexal skin tumors caused by germ line mutations in the CYLD gene. In most cases the second wild type allele is lost in tumor tissue suggesting that CYLD functions as tumor-suppressor. CYLD is a protein of 956 amino acids harboring a functional deubiquitinating domain at the C-terminal end. CYLD was shown to interact with TRAF2, NEMO and TRIP. Furthermore, CYLD is a negative regulator of NF-kappaB activation mediated by TNF family members and IL-1beta. Downregulation of NF-kappaB activity depends on the deubiquitinating activity of CYLD which removes lysine-63 linked ubiquitin chains from target proteins. These findings indicate that cylindromas arise through constitutive NF-kappaB activation leading to hyperproliferation and tumor growth. In summary, CYLD contributes to the regulation of cell survival, proliferation and differentiation via its effects on NF-kappaB activation. It is a negative regulator of the Wnt signaling, inhibits HDAC6 and thereby promotes acetylation of alpha-tubulin and stabilization of microtubules. Through the regulation of microtubule dynamics, it contributes to the regulation of cell proliferation, cell polarization, cell migration and angiogenesis. It plays a role in the regulation of inflammation and the innate immune response via its effects on NF-kappaB activation. Defects in CYLD are the cause i) of familial cylindromatosis (FCYL), also known as Ancell-Spiegler cylindromas or turban tumor, ii) of multiple familial trichoepithelioma type 1 (MFT1), also known as epithelioma adenoides cysticum of Brooke (EAC) and iii) of Brooke-Spiegler syndrome (BRSS), all characterized by the presence of many skin tumors. |
Immunogen | Synthetic peptide corresponding to aa 56-70 (G56HSRIPSAKGKKNQI70) of human CYLD. |
Other Names | cyld|2010013M14Rik|2900009M21Rik|C130039D01Rik|CDMT|CYLD1|EAC|mKIAA0849|LRRGT00003|Rp1|Rp1h|BRSS|CYLDI|MFT|MFT1|SBS|TEM|USPL2 |
Gene, Accession # | Gene ID: 1540 |
Catalog # | ABIN1169139 |
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Order / More Info | Cylindromatosis (Turban Tumor Syndrome) (CYLD) (aa 56-70) Antibody from ANTIBODIES-ONLINE GmbH |
Product Specific References | n/a |
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