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anti-AAAS antibody :: Rabbit AAAS Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS2528726
Immunohistochemistry (IHC)
Unit / Price
0.02 mL  /  $110 +1 FREE 8GB USB
0.06 mL  /  $150 +1 FREE 8GB USB
0.12 mL  /  $225 +1 FREE 8GB USB
0.2 mL  /  $360 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

AAAS, Polyclonal Antibody

 Also Known As   

AAAS Polyclonal Antibody

 Product Synonym Names    AAA; AAASb; GL003; ALADIN; ADRACALA; ADRACALIN
 Product Gene Name   

anti-AAAS antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    231550
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Antigen affinity purification
 Concentration    1.2mg/mL (lot specific)
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 Immunogen    Recombinant protein of human AAAS
 Buffer    PBS with 0.05% sodium azide, 50% glycerol, pH7.3
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 Preparation and Storage    Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.
 Other Notes    Small volumes of anti-AAAS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-AAAS antibody

   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.
 Applications Tested/Suitable for anti-AAAS antibody   

ELISA (EIA), Immunohistochemistry (IHC)

 Application Notes for anti-AAAS antibody    IHC: 1:25-1:100
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 Immunohistochemistry (IHC) of anti-AAAS antibody    Immunohistochemistry of Human brain using AAAS Polyclonal Antibody at dilution of 1:20
anti-AAAS antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for AAAS. It may not necessarily be applicable to this product.
 NCBI GI #    291045307
 NCBI GeneID    8086
 NCBI Accession #    NP_001166937.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001173466.1 [Other Products]
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 UniProt Secondary Accession #    Q5JB47; Q9NWI6; Q9UG19 [Other Products]
 UniProt Related Accession #    Q9NRG9 [Other Products]
 Molecular Weight    55,814 Da
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 NCBI Official Full Name    aladin isoform 2
 NCBI Official Synonym Full Names    achalasia, adrenocortical insufficiency, alacrimia
 NCBI Official Symbol    AAAS [Similar Products]
 NCBI Official Synonym Symbols   
AAA; AAASb; GL003; ALADIN; ADRACALA; ADRACALIN
[Similar Products]
 NCBI Protein Information    aladin; Allgrove, triple-A
 UniProt Protein Name    Aladin
 UniProt Synonym Protein Names   
Adracalin
 Protein Family    Aladin
 UniProt Gene Name    AAAS [Similar Products]
 UniProt Synonym Gene Names    ADRACALA [Similar Products]
 UniProt Entry Name    AAAS_HUMAN
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 NCBI Summary for AAAS    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]
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 UniProt Comments for AAAS    AAAS: Plays a role in the normal development of the peripheral and central nervous system. Defects in AAAS are the cause of achalasia-addisonianism- alacrima syndrome (AAAS); 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. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Nuclear envelope; Membrane protein, integral

Chromosomal Location of Human Ortholog: 12q13

Cellular Component: nucleoplasm; centrosome; nuclear membrane; membrane; cytoplasm; nuclear pore; nuclear envelope; nucleus

Biological Process: mRNA transport; viral reproduction; cytokine and chemokine mediated signaling pathway; mitotic nuclear envelope disassembly; pathogenesis; nucleocytoplasmic transport; learning; viral infectious cycle; glucose transport; protein transport; fertilization; hexose transport; regulation of nucleocytoplasmic transport; carbohydrate metabolic process; viral transcription; gene expression; mitotic cell cycle; transmembrane transport

Disease: Achalasia-addisonianism-alacrima Syndrome
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 Research Articles on AAAS    1. Data suggest ALADIN is involved in resistance to oxidative stress in adrenocortical cells/neurons; ALADIN knockdown down-regulates StAR (steroidogenic acute regulatory protein) and P45011beta (cytochrome P450 family 11 subfamily B polypeptide 1).
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Pathways associated with anti-AAAS antibodyDiseases associated with anti-AAAS antibody
 Products by Pathway  Pathway Diagram
 Antiviral Mechanism By IFN-stimulated Genes Pathway antibodies  Antiviral Mechanism By IFN-stimulated Genes Pathway Diagram
 Cell Cycle Pathway antibodies  Cell Cycle Pathway Diagram
 Cell Cycle, Mitotic Pathway antibodies  Cell Cycle, Mitotic Pathway Diagram
 Cytokine Signaling In Immune System Pathway antibodies  Cytokine Signaling In Immune System Pathway Diagram
 Disease Pathway antibodies  Disease Pathway Diagram
 Export Of Viral Ribonucleoproteins From Nucleus Pathway antibodies  Export Of Viral Ribonucleoproteins From Nucleus Pathway Diagram
 Gene Expression Pathway antibodies  Gene Expression Pathway Diagram
 Glucose Transport Pathway antibodies  Glucose Transport Pathway Diagram
 Glycogen Storage Diseases Pathway antibodies  Glycogen Storage Diseases Pathway Diagram
 HIV Infection Pathway antibodies  HIV Infection Pathway Diagram
 Disease Name  Pubmed Publications
 Inflammation Antibodies  >165 publications with AAAS and Inflammation
 Kidney Diseases Antibodies  >43 publications with AAAS and Kidney Diseases
 Necrosis Antibodies  >39 publications with AAAS and Necrosis
 Congenital Abnormalities Antibodies  >35 publications with AAAS and Congenital Abnormalities
 Achalasia Addisonianism Alacrimia syndrome Antibodies  >18 publications with AAAS and Achalasia Addisonianism Alacrimia syndrome
 Fibrosis Antibodies  >15 publications with AAAS and Fibrosis
 Liver Diseases Antibodies  >15 publications with AAAS and Liver Diseases
 Abnormalities, Multiple Antibodies  >14 publications with AAAS and Abnormalities, Multiple
 Weight Loss Antibodies  >7 publications with AAAS and Weight Loss
 Bone Diseases, Developmental Antibodies  >4 publications with AAAS and Bone Diseases, Developmental
Organs/Tissues associated with anti-AAAS antibody
 Organ/Tissue Name  Pubmed Publications
 Vascular Antibodies  >1175 publications with AAAS and Vascular
 Blood Antibodies  >766 publications with AAAS and Blood
 Muscle Antibodies  >147 publications with AAAS and Muscle
 Heart Antibodies  >101 publications with AAAS and Heart
 Nerve Antibodies  >78 publications with AAAS and Nerve
 Kidney Antibodies  >53 publications with AAAS and Kidney
 Skin Antibodies  >39 publications with AAAS and Skin
 Connective Tissue Antibodies  >33 publications with AAAS and Connective Tissue
 Bone Antibodies  >31 publications with AAAS and Bone
 Eye Antibodies  >29 publications with AAAS and Eye
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