FITC標記的Allgrove綜合征相關蛋白抗體

FITC標記的Allgrove綜合征相關蛋白抗體
英文名稱：Anti-Adracalin/FITC
中文名稱別名：AAA; AAAS; AAASb; Achalasia adrenocortical insufficiency alacrimia (Allgrove triple A); Achalasia adrenocortical insufficiency alacrimia; ADRACALA; Aladin; Allgrove triple A; DKFZp586G1624; GL003; AAAS_HUMAN.
規(guī)格：100ul
研究領域：細胞生物   免疫學   發(fā)育生物學   神經生物學    細胞類型標志物
抗體來源  ：兔
克隆類型：多克隆
標記一抗訂購
交叉反應：Human, Mouse, Rat, Cow,
產品應用：ICC = 1：50-200 IF = 1：50-200
在其他應用程序尚未進行測試。
最佳稀釋/濃度：應該由最終用戶來確定。
分子量:約為60kDa
性狀：凍干或液體
濃度：為1mg / ml
亞型IgG抗體
純化方法：affinity purified by Protein A
儲存液防腐劑：15毫米的疊氮化鈉，成分：1％BSA，0.01M的PBS，pH 7.4中
保存條件：儲存在-20℃一年。避免反復冷凍/解凍循環(huán)。當保持在-20℃下凍干抗體是在室溫下穩(wěn)定至少一個月，對于大于一年。當在無菌的pH 7.4的0.01M PBS或抗體的稀釋液重構的抗體可穩(wěn)定至少兩周，在2-4℃。
產品圖片：

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.

原創(chuàng)作者：青島捷世康生物科技有限公司

標簽：FITC標記的Allgrove綜合征相關蛋白抗體 Anti-Adracalin/FITC 

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