ITT5042
ITT5042
ITT5042
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- Catalog: ITT5042
- Gene/Protein: AAAS
- Product Description: Immunotag™ Aladin Polyclonal Antibody
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Description
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein Aladin Clonality Polyclonal Storage/Stability -20°C/1 year Application WB,ELISA Recommended Dilution Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications. Concentration 1 mg/ml Reactive Species Human,Rat Host Species Rabbit Immunogen Synthesized peptide derived from Aladin . at AA range: 360-440 Specificity Aladin Polyclonal Antibody detects endogenous levels of Aladin protein. Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Gene Name AAAS Accession No. Q9NRG9 P58742 Alternate Names AAAS; ADRACALA; GL003; Aladin; Adracalin Description aladin WD repeat nucleoporin(AAAS) Homo sapiens The protein encoded by this gene is a member of the WD-repeat family of regulatory proteins and may be involved in normal development of the peripheral and central nervous system. The encoded protein is part of the nuclear pore complex and is anchored there by NDC1. Defects in this gene are a cause of achalasia-addisonianism-alacrima syndrome (AAAS), also called triple-A syndrome or Allgrove syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2010], Protein Expression Adipose tissue,Kidney,Liver,Uterus, Subcellular Localization nucleus,nuclear envelope,nuclear pore,nucleoplasm,cytoplasm,centrosome,membrane,nuclear membrane, Protein Function 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.,function:Plays a role in the normal development of the peripheral and central nervous system.,similarity:Contains 4 WD repeats.,tissue specificity:Widely expressed. Particularly abundant expression is found in cerebellum, corpus callosum, adrenal gland, pituary gland, gatsrointestinal structures and fetal lung., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | Aladin |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Rat |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from Aladin . at AA range: 360-440 |
| Specificity | Aladin Polyclonal Antibody detects endogenous levels of Aladin protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | AAAS |
| Accession No. | Q9NRG9 P58742 |
| Alternate Names | AAAS; ADRACALA; GL003; Aladin; Adracalin |
| Description | aladin WD repeat nucleoporin(AAAS) Homo sapiens The protein encoded by this gene is a member of the WD-repeat family of regulatory proteins and may be involved in normal development of the peripheral and central nervous system. The encoded protein is part of the nuclear pore complex and is anchored there by NDC1. Defects in this gene are a cause of achalasia-addisonianism-alacrima syndrome (AAAS), also called triple-A syndrome or Allgrove syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2010], |
| Protein Expression | Adipose tissue,Kidney,Liver,Uterus, |
| Subcellular Localization | nucleus,nuclear envelope,nuclear pore,nucleoplasm,cytoplasm,centrosome,membrane,nuclear membrane, |
| Protein Function | 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.,function:Plays a role in the normal development of the peripheral and central nervous system.,similarity:Contains 4 WD repeats.,tissue specificity:Widely expressed. Particularly abundant expression is found in cerebellum, corpus callosum, adrenal gland, pituary gland, gatsrointestinal structures and fetal lung., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |