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Rabbit Anti-GFAP/BF594 Conjugated antibody (bs-0199R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-0199R-BF594
英文名稱 Rabbit Anti-GFAP/BF594 Conjugated antibody
中文名稱 BF594標記的膠質纖維酸性蛋白抗體
別    名 Astrocyte; FLJ45472; GFAP; Glial Fibrillary Acidic Protein; Intermediate filament protein; GFAP_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  免疫學  神經生物學  信號轉導  干細胞  細胞粘附分子  細胞類型標志物  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Dog, Pig, Cow, Rabbit, Sheep, )
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 48kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GFAP
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:
This gene encodes one of the major intermediate filament proteins of mature astrocytes. It is used as a marker to distinguish astrocytes from other glial cells during development. Mutations in this gene cause Alexander disease, a rare disorder of astrocytes in the central nervous system. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Oct 2008]

Function:
GFAP, a class-III intermediate filament, is a cell-specific marker that, during the development of the central nervous system, distinguishes astrocytes from other glial cells.

Subunit:
Interacts with SYNM. Isoform 3 interacts with PSEN1 (via N-terminus).

Subcellular Location:
Cytoplasm. Note=Associated with intermediate filaments.

Tissue Specificity:
Expressed in cells lacking fibronectin.

Post-translational modifications:
Phosphorylated by PKN1.

DISEASE:
Defects in GFAP are a cause of Alexander disease (ALEXD) [MIM:203450]. Alexander disease is a rare disorder of the central nervous system. It is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of Rosenthal fibers which are cytoplasmic inclusions in astrocytes. The most common form affects infants and young children, and is characterized by progressive failure of central myelination, usually leading to death usually within the first decade. Infants with Alexander disease develop a leukoencephalopathy with macrocephaly, seizures, and psychomotor retardation. Patients with juvenile or adult forms typically experience ataxia, bulbar signs and spasticity, and a more slowly progressive course.

Similarity:
Belongs to the intermediate filament family.

Database links:

Entrez Gene: 281189 Cow

Entrez Gene: 2670 Human

Entrez Gene: 14580 Mouse

Entrez Gene: 24387 Rat

Omim: 137780 Human

SwissProt: Q28115 Cow

SwissProt: P14136 Human

SwissProt: P03995 Mouse



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

星形膠質細胞標志物 (Astrocyte Marker)

GFAP是一個56kDa的中間絲蛋白(intermediate filament,IF),在中樞神經系統發育期是一個特異性的標志物,以區別星形細胞和其它膠質細胞。GFAP表達在皮層和海馬,急、慢性皮質酮治療時表達減少。
GFAP可以和人、大鼠、小鼠的GFAP反應,在正常和腫瘤性的星形膠質細胞陽性表達,而神經節細胞、神經元、成纖維細胞、少突膠質細胞和這些細胞來源的腫瘤細胞陰性表達,主要用于星形膠質瘤等中樞神經系統腫瘤的診斷和鑒別診斷,GFAP的缺乏可導致AD病。
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