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CYLD Mouse mAb (bsm-51375M)  
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產(chǎn)品編號(hào) bsm-51375M
英文名稱 CYLD Mouse mAb
中文名稱 微管結(jié)合蛋白CYLD單克隆抗體
別    名 CDMT; cylindromatosis(turban tumor syndrome); cylindromatosis 1; Deubiquitinating enzyme CYLD; EAC; HSPC057; KIAA0849; turban tumor syndrome; Ubiquitin thiolesterase CYLD; Ubiquitin-specific processing protease CYLD; CYLD_HUMAN; Ubiquitin carboxyl-termina  
研究領(lǐng)域 腫瘤  細(xì)胞生物  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號(hào) 5C7
交叉反應(yīng) Human
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:50-200,IF=1:50-200,ELISA=1:1000-5000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 105 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CYLD 
亞    型 IgG1
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 Defects in CYLD are the cause of familial cylindromatosis (CYLD) also known as turban tumor syndrome or dermal eccrine cylindromatosis. CYLD is an autosomal dominant and highly tumor type-specific disorder. The tumors (known as cylindromas because of their characteristic microscopic architecture) are believed to arise from or recapitulate the appearance of the eccrine or apocrine cells of the skin that secrete sweat and scent respectively. Cylindromas arise predominantly in hairy parts of the body with approximately 90% on the head and neck. The development of a confluent mass which may ulcerate or become infected has led to the designation "turban tumor syndrome". The skin tumors show differentiation in the direction of hair structures, hence the synonym trichoepithelioma. CYLD has deubiquitinating activity.

Function:
Protease that specifically cleaves 'Lys-63'-linked polyubiquitin chains. Has endodeubiquitinase activity. Plays an important role in the regulation of pathways leading to NF-kappa-B activation. Contributes to the regulation of cell survival, proliferation and differentiation via its effects on NF-kappa-B activation. Negative regulator of Wnt signaling. Inhibits HDAC6 and thereby promotes acetylation of alpha-tubulin and stabilization of microtubules. Plays a role in the regulation of microtubule dynamics, and thereby contributes to the regulation of cell proliferation, cell polarization, cell migration, and angiogenesis. Required for normal cell cycle progress and normal cytokinesis. Inhibits nuclear translocation of NF-kappa-B. Plays a role in the regulation of inflammation and the innate immune response, via its effects on NF-kappa-B activation. Dispensable for the maturation of intrathymic natural killer cells, but required for the continued survival of immature natural killer cells. Negatively regulates TNFRSF11A signaling and osteoclastogenesis

Subunit:
Interacts (via CAP-Gly domain) with IKBKG/NEMO (via proline-rich C-terminal region). Interacts with TRAF2 and TRIP. Interacts with PLK1, DVL1, DVL3, MAVS, TBK1, IKKE and DDX58. Interacts (via CAP-Gly domain) with microtubules. Interacts with HDAC6 and BCL3. Interacts with SQSTM1 and MAP3K7. Identified in a complex with TRAF6 and SQSTM1.

Subcellular Location:
Cytoplasm. Cytoplasm, perinuclear region. Cytoplasm, cytoskeleton. Cell membrane; Peripheral membrane protein; Cytoplasmic side. Note=Detected at the microtubule cytoskeleton during interphase. Detected at the midbody during telophase.

Tissue Specificity:
Detected in fetal brain, testis, and skeletal muscle, and at a lower level in adult brain, leukocytes, liver, heart, kidney, spleen, ovary and lung. Isoform 2 is found in all tissues except kidney.

Post-translational modifications:
Phosphorylated on several serine residues by IKKA and/or IKKB in response to immune stimuli. Phosphorylation requires IKBKG. Phosphorylation abolishes TRAF2 deubiquitination, interferes with the activation of Jun kinases, and strongly reduces CD40-dependent gene activation by NF-kappa-B.

DISEASE:
Familial cylindromatosis (FCYL) [MIM:132700]: Autosomal dominant and highly tumor type-specific disorder. The tumors (known as cylindromas because of their characteristic microscopic architecture) are believed to arise from or recapitulate the appearance of the eccrine or apocrine cells of the skin that secrete sweat and scent respectively. Cylindromas arise predominantly in hairy parts of the body with approximately 90% on the head and neck. The development of a confluent mass which may ulcerate or become infected has led to the designation 'turban tumor syndrome'. The skin tumors show differentiation in the direction of hair structures, hence the synonym trichoepithelioma. Note=The disease is caused by mutations affecting the gene represented in this entry.
Multiple familial trichoepithelioma 1 (MFT1) [MIM:601606]: Autosomal dominant dermatosis characterized by the presence of many skin tumors predominantly on the face. Since histologic examination shows dermal aggregates of basaloid cells with connection to or differentiation toward hair follicles, this disorder has been thought to represent a benign hamartoma of the pilosebaceous apparatus. Trichoepitheliomas can degenerate into basal cell carcinoma. Note=The disease is caused by mutations affecting the gene represented in this entry.
Brooke-Spiegler syndrome (BRSS) [MIM:605041]: An autosomal dominant disorder characterized by the appearance of multiple skin appendage tumors such as cylindroma, trichoepithelioma, and spiradenoma. These tumors are typically located in the head and neck region, appear in early adulthood, and gradually increase in size and number throughout life. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the peptidase C67 family.
Contains 3 CAP-Gly domains.

SWISS:
Q9NQC7

Gene ID:
1540

Database links:

Entrez Gene: 536421 Cow

Entrez Gene: 1540 Human

Entrez Gene: 74256 Mouse

Entrez Gene: 312937 Rat

Omim: 605018 Human

SwissProt: Q1RMU2 Cow

SwissProt: Q9NQC7 Human

SwissProt: Q80TQ2 Mouse

SwissProt: Q66H62 Rat

Unigene: 578973 Human

Unigene: 482446 Mouse

Unigene: 128760 Rat

Unigene: 168938 Rat



產(chǎn)品圖片
25 ug total protein per lane of various lysates (see on figure) probed with CYLD monoclonal antibody, unconjugated (bsm-51375M) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
Paraformaldehyde-fixed, paraffin embedded Human Brain; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with CYLD Monoclonal Antibody, Unconjugated(bsm-51375M) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human testicles; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with CYLD Monoclonal Antibody, Unconjugated(bsm-51375M) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
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