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Alternative name
AAA, AAAS, AAASb, ADRACALA, ADRACALIN, ALADIN, DKFZp586G1624, GL003 antibody
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Catalog
A000275
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Size100μg
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Formliquid
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Purity≥95% as determined by SDS-PAGE
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StoragePBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 24 months (Avoid repeated freeze / thaw cycles.)
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ClonalityPolyclonal Antibody
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Host Rabbit
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Species ReactivityHuman
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Applications Tested/SuitableELISA,WB,IHC,IF
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PurificationImmunogen affinity purified
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Immunogenachalasia, adrenocortical insufficiency, alacrimia (Allgrove, triple-A)
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IHCImmunohistochemistry of paraffin-embedded human stomach cancer using A000275(AAAS antibody) at dilution of 1:100
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Western BlotSH-SY5Y cells were subjected to SDS PAGE followed by western blot with A000275(AAAS antibody) at dilution of 1:600
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Recommended dilutionWB :1:500-1:5000;IHC:1:20-1:200;IF:1:20-1:200
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Product Description specificalThe AAAS gene product is the 60 kDa nuclear pore complex (NPC) protein alacrima-achalasia-adrenal insufficiency neurological disorder (ALADIN). AAAS mRNA and the ALADIN protein are ubiquitously expressed with predominance in the adrenal and central nervous system structures in human and rat. ALADIN, a Tryptophan-Aspartic acid (WD) repeat containing protein, was the first nuclear pore complex protein to be associated with hereditary neurodegenerative disease and the only nuclear pore complex protein to be associated with hereditary adrenal disease. ALADIN's precise function at the nuclear pore complex is unknown. Most naturally occurring AAAS mutations result in mislocalization of the abnormal ALADIN protein (mainly into the cytoplasm), implying that correct NPC targeting is vital for its function. (PMID: 23825130)
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Gene nameAAAS
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locationNuclear pore complex,Nucleus
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Uniprot IDQ9NRG9
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Calculated M.W.60kDa
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Observed M.W.60 kDa
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