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Rabbit Anti-SPG11/BF594 Conjugated antibody (bs-17656R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-17656R-BF594
英文名稱1 Rabbit Anti-SPG11/BF594 Conjugated antibody
中文名稱 BF594標記的大腸癌相關蛋白抗體
別    名 Colorectal carcinoma associated protein; Colorectal carcinoma-associated protein; DKFZp762B1512; FLJ21439; KIAA1840; Spastic paraplegia 11 (autosomal recessive); Spastic paraplegia 11; Spastic paraplegia 11 protein; Spatacsin; SPG 11; Spg11; SPTCS_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse,  (predicted: Dog, Pig, 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 279kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SPG11
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 protein encoded by this gene is a potential transmembrane protein that is phosphorylated upon DNA damage. Defects in this gene are a cause of spastic paraplegia type 11 (SPG11). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2009]

Subunit:
Interacts with AP5Z1, AP5B1, AP5S1 and ZFYVE26.

Subcellular Location:
Membrane. Cytoplasm > cytosol. Nucleus. Mainly cytoplasmic.

Tissue Specificity:
Expressed in all structures of brain, with a high expression in cerebellum.

Post-translational modifications:
Phosphorylated upon DNA damage, probably by ATM or ATR.

DISEASE:
Defects in SPG11 are the cause of spastic paraplegia autosomal recessive type 11 (SPG11) [MIM:604360]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.

Database links:

Entrez Gene: 80208 Human

Omim: 610844 Human

SwissProt: Q96JI7 Human

Unigene: 656271 Human

Unigene: 683876 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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