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Rabbit Anti-KBTBD13/PE-Cy7 Conjugated antibody (bs-17086R-PE-Cy7)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-17086R-PE-Cy7
英文名稱 Rabbit Anti-KBTBD13/PE-Cy7 Conjugated antibody
中文名稱 PE-Cy7標記的KBTBD13蛋白抗體
別    名 5430433E21Rik; HCG1645727; KBTBD_HUMAN; Kbtbd13; Kelch repeat and BTB (POZ) domain containing 13; Kelch repeat and BTB domain-containing protein 13; NEM6.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  發育生物學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Cow, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 49kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KBTBD13
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
The gene belongs to a family of genes encoding proteins containing a BTB domain and several kelch repeats. The BTB domain functions as a protein-protein interaction module, which includes an ability to self-associate or to interact with non-BTB domain-containing proteins. The kelch motif typically occurs in groups of five to seven repeats, and has been found in proteins with diverse functions. Known functions of these family members include transcription regulation, ion channel tetramerization and gating, protein ubiquitination or degradation, and cytoskeleton regulation. The exact function of this family member has yet to be determined. [provided by RefSeq, Jun 2010]

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Expressed in skeletal muscle.

DISEASE:
Defects in KBTBD13 are the cause of nemaline myopathy type 6 (NEM6) [MIM:609273]. A form of nemaline myopathy characterized by childhood onset of slowly progressive proximal muscle weakness, exercise intolerance, and slow movements with stiff muscles. Patients are unable to run or correct themselves from falling over.

Similarity:
Contains 1 BTB (POZ) domain.
Contains 5 Kelch repeats.

Database links:

Entrez Gene: 390594 Human

Omim: 613727 Human

SwissProt: C9JR72 Human

Unigene: 586890 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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