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phospho-CYLD (Ser418) Rabbit pAb (bs-3080R)  
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產品編號 bs-3080R
英文名稱 phospho-CYLD (Ser418) Rabbit pAb
中文名稱 磷酸化微管結合蛋白CYLD抗體
別    名 CDMT; cylindromatosis(turban tumor syndrome); Deubiquitinating enzyme CYLD; EAC; HSPC057; KIAA0849; turban tumor syndrome; Ubiquitin thiolesterase CYLD; Ubiquitin-specific processing protease CYLD; Cyld; CYLD_HUMAN; cylindromatosis 1; Ubiquitin-specific processing protease CYLD; Ubiquitin-specific-processing protease CYLD.  
產品類型 磷酸化抗體 
研究領域 腫瘤  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse (predicted: Human,Rat,Pig,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 105 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated Synthesised phosphopeptide derived from human CYLD around the phosphorylation site of Ser418: FH(p-S)LP 
亞    型 IgG
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 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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 (By similarity).

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:
Defects in CYLD are the cause of familial cylindromatosis (FCYL) [MIM:132700]; also known as Ancell-Spiegler cylindromas or 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. Defects in CYLD are the cause of multiple familial trichoepithelioma type 1 (MFT1) [MIM:601606]; also known as epithelioma adenoides cysticum of Brooke (EAC) or hereditary multiple benign cystic epithelioma or Brooke-Fordyce trichoepitheliomas. MFT1 is an 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. Defects in CYLD are the cause of Brooke-Spiegler syndrome (BRSS) [MIM:605041]. BRSS is 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.

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

SWISS:
Q9NQC7

Gene ID:
1540

Database links:

Entrez Gene: 1540 Human

Entrez Gene: 536421 Cow

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



????CYLD(cylindromatosis)是近年發現的一種腫瘤抑制基因,CYLD丟失或突變可致腫瘤形成,多個研究顯示,其表達蛋白CYLD可去泛素化TRAFs、NEMO、Bcl-3及p53等信號分子,調控細胞NF-κB和JNK等信號途徑,CYLD在人體內廣泛分布,在細胞周期的調控、介導細胞凋亡、抑制腫瘤發生等分子事件中有著重要的調控作用。
????CYLD基因缺陷或缺失主要導致頭部或面部的皮膚腫瘤,如多發性家族性毛發上皮瘤(MFT),家族性圓柱瘤(FC)和Brooke-Spiegler綜合癥(BSS),此外,CYLD在宮頸癌、腎癌、結腸癌、肝癌形成的信號途徑中也均表現為下游調控,是目前用于腫瘤研究的熱門抗體。
產品圖片
Sample: Lane1: Heart (Mouse) Lysate at 30 ug Lane2: Sp2/0 (Mouse) Cell Lysate at 30 ug Primary: Anti-phospho-CYLD(Ser418) (bs-3080R) at 1:200 dilution; Secondary: HRP conjugated Goat Anti-Rabbit IgG(bs-0295G-HRP) at 1: 3000 dilution; Predicted band size : 105kD Observed band size : 105kD
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