CYLD (Cylindromatosis Tumor Suppressor Protein)

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.\n\nApplications:\nSuitable for use in Immunocytochemistry, Western Blot\n\nRecommended Dilution:\nOptimal dilutions to be determined by researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

044719

Size

50ug

Applications

ICC, WB

Hosts

Rabbit

Form

Supplied as a liquid in PBS, 1% BSA, 50% glycerol, 0.02% sodium azide.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

References

Product Reference: \n?The tumor suppressor CYLD Acts as a Negative Regulator for Toll-like Receptor 2 Signaling via Negative Cross-talk with TRAF6 and TRAF7: H. Yoshida, et al.; J. Biol. Chem. 280, 41111 (2005)\n?

Additional Info

Recognizes human CYLD. Detects a band of ~120kD by Western blot.

Alternative Names

Cylindromatosis Tumor Suppressor Protein