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Rabbit Anti-Adracalin/BF350 Conjugated antibody (bs-11807R-BF350)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11807R-BF350
英文名稱 Rabbit Anti-Adracalin/BF350 Conjugated antibody
中文名稱 BF350標記的Allgrove綜合征相關蛋白抗體
別    名 AAA; AAAS; AAASb; Achalasia adrenocortical insufficiency alacrimia (Allgrove triple A); Achalasia adrenocortical insufficiency alacrimia; ADRACALA; Aladin; Allgrove triple A; DKFZp586G1624; GL003; AAAS_HUMAN.  
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  神經生物學  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, 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 60kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Adracalin
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:
Aladin (Adracalin) belongs to a family of WD repeat-containing proteins. These proteins have a wide variety of functions, including signal transduction regulation, RNA processing and transcription. Aladin plays a role in peripheral and central nervous system development. It is widely expressed, with the highest expression seen in pituitary gland, corpus callosum, cerebellum, adrenal gland and gastrointestinal structures. Defects in Aladin cause the autosomal recessive disorder achalasia-addisonianism-alacrima (triple A) syndrome. Triple A syndrome is characterized by achalasia, alacrima and adrenocortico-tropin-resistant adrenal insufficiency. Robust expression in neural systems associated with cognitive, motor and sensory functions is consistent with the myriad of symptoms experienced by patients with triple A syndrome.

Function:
Adracalin (AAAS) is expressed in both neuroendocrine and cerebral structures and may function in the normal development of the peripheral and central nervous system. It localizes to nuclear pore complexes (NPCs), large multiprotein assemblies that are the sole sites of nucleocytoplasmic transport. Defects in AAAS are the cause of achalasia-addisonianism-alacrima syndrome (AAA syndrome); also known as triple-A syndrome or Allgrove syndrome.

Subcellular Location:
nuclear pore

Tissue Specificity:
Widely expressed. Particularly abundant expression is found in cerebellum, corpus callosum, adrenal gland, pituitary gland, gastrointestinal structures and fetal lung.

DISEASE:
Defects in AAAS are the cause of achalasia-addisonianism-alacrima syndrome (AAAS) [MIM:231550]; also known as triple-A syndrome or Allgrove syndrome. AAAS is an autosomal recessive disorder characterized by adreno-corticotropic hormone (ACTH)-resistant adrenal failure, achalasia of the esophageal cardia and alacrima. The syndrome is associated with variable and progressive neurological impairment involving the central, peripheral, and autonomic nervous system. Other features such as palmoplantar hyperkeratosis, short stature, facial dysmorphy and osteoporosis may also be present.

Similarity:
Contains 4 WD repeats.

Database links:
UniProtKB/Swiss-Prot: Q9NRG9.1

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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