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SQSTM1/P62 Mouse mAb (bsm-51287M)  
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50ul/1180.00元
100ul/1980.00元
大包裝/詢價
產品編號 bsm-51287M
英文名稱 SQSTM1/P62 Mouse mAb
中文名稱 SQSTM1/P62單克隆抗體
別    名 SQSTM_HUMAN; Sequestosome-1; ORCA; OSIL; EBI3-associated protein of 60 kDa(EBIAP; p60); phosphotyrosine-independent ligand for the Lck SH2 domain of 62 kDa; Ubiquitin-binding protein p62;  
研究領域 心血管  染色質和核信號  信號轉導  細胞凋亡  轉錄調節因子  表觀遺傳學  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 6C5
交叉反應 (predicted: Human)
產品應用 WB=1:500-2000,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 60 kDa
檢測分子量
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SQSTM1/P62 
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 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 This gene encodes a multifunctional protein that binds ubiquitin and regulates activation of the nuclear factor kappa-B (NF-kB) signaling pathway. The protein functions as a scaffolding/adaptor protein in concert with TNF receptor-associated factor 6 to mediate activation of NF-kB in response to upstream signals. Alternatively spliced transcript variants encoding either the same or different isoforms have been identified for this gene. Mutations in this gene result in sporadic and familial Paget disease of bone. [provided by RefSeq, Mar 2009]

Function:
Adapter protein which binds ubiquitin and may regulate the activation of NFKB1 by TNF-alpha, nerve growth factor (NGF) and interleukin-1. May play a role in titin/TTN downstream signaling in muscle cells. May regulate signaling cascades through ubiquitination. Adapter that mediates the interaction between TRAF6 and CYLD (By similarity). May be involved in cell differentiation, apoptosis, immune response and regulation of K(+) channels.

Subunit:
Cytoplasm. Late endosome. Nucleus. Endoplasmic reticulum. Note=Sarcomere. In cardiac muscles localizes to the sarcomeric band. Localizes to late endosomes. May also localize to the nucleus. Accumulates in neurofibrillary tangles and in Lewy bodies of neurons from individuals with Alzheimer and Parkinson disease respectively. Enriched in Rosenthal fibers of pilocytic astrocytoma. In liver cells, accumulates in Mallory bodies associated with alcoholic hepatitis, Wilson disease, indian childhood cirrhosis and in hyaline bodies associated with hepatocellular carcinoma. Colocalizes with TRIM13 in the perinuclear endoplasmic reticulum.

Subcellular Location:
Cytoplasm. Late endosome. Nucleus. Sarcomere (By similarity). In cardiac muscles localizes to the sarcomeric band (By similarity). Localizes to late endosomes. May also localize to the nucleus. Accumulates in neurofibrillary tangles and in Lewy bodies of neurons from individuals with Alzheimer and Parkinson disease respectively. Enriched in Rosenthal fibers of pilocytic astrocytoma. In liver cells, accumulates in Mallory bodies associated with alcoholic hepatitis, Wilson disease, indian childhood cirrhosis and in hyaline bodies associated with hepatocellular carcinoma.

Tissue Specificity:
Ubiquitously expressed.

Post-translational modifications:
Phosphorylated. May be phosphorylated by PRKCZ (By similarity). Phosphorylated in vitro by TTN.

DISEASE:
Defects in SQSTM1 are a cause of Paget disease of bone (PDB) [MIM:602080]. PDB is a metabolic bone disease affecting the axial skeleton and characterized by focal areas of increased and disorganized bone turn-over due to activated osteoclasts. Manifestations of the disease include bone pain, deformity, pathological fractures, deafness, neurological complications and increased risk of osteosarcoma. PDB is a chronic disease affecting 2 to 3% of the population above the age of 40 years.

Similarity:
Contains 1 OPR domain.
Contains 1 UBA domain.
Contains 1 ZZ-type zinc finger.

SWISS:
Q13501

Gene ID:
8878

Database links:

Entrez Gene: 8878 Human

SwissProt: Q13501 Human



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