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Rabbit Anti-GFAP/BF647 Conjugated antibody (bs-0199R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-0199R-BF647
英文名稱 Rabbit Anti-GFAP/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的膠質(zhì)纖維酸性蛋白抗體
別    名 Astrocyte; FLJ45472; GFAP; Glial Fibrillary Acidic Protein; Intermediate filament protein; GFAP_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  干細(xì)胞  細(xì)胞粘附分子  細(xì)胞類型標(biāo)志物  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat,  (predicted: Dog, Pig, Cow, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 Flow-Cyt=1:50-200 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(chǔ) 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(chǎn)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(zhì)細(xì)胞標(biāo)志物 (Astrocyte Marker)

GFAP是一個(gè)56kDa的中間絲蛋白(intermediate filament,IF),在中樞神經(jīng)系統(tǒng)發(fā)育期是一個(gè)特異性的標(biāo)志物,以區(qū)別星形細(xì)胞和其它膠質(zhì)細(xì)胞。GFAP表達(dá)在皮層和海馬,急、慢性皮質(zhì)酮治療時(shí)表達(dá)減少。
GFAP可以和人、大鼠、小鼠的GFAP反應(yīng),在正常和腫瘤性的星形膠質(zhì)細(xì)胞陽性表達(dá),而神經(jīng)節(jié)細(xì)胞、神經(jīng)元、成纖維細(xì)胞、少突膠質(zhì)細(xì)胞和這些細(xì)胞來源的腫瘤細(xì)胞陰性表達(dá),主要用于星形膠質(zhì)瘤等中樞神經(jīng)系統(tǒng)腫瘤的診斷和鑒別診斷,GFAP的缺乏可導(dǎo)致AD病。
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