Full Product Name
AAAS Antibody - N-terminal region
Product Gene Name
anti-AAAS antibody
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Product Synonym Gene Name
AAAS; ADRACALA; GL003;[Similar Products]
Antibody/Peptide Pairs
AAAS peptide (MBS3244065) is used for blocking the activity of AAAS antibody (MBS3219173)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence
Synthetic peptide located within the following region: EHNNELVTGS SYESPPPDFR GQWINLPVLQ LTKDPLKTPG RLDHGTRTAF
3D Structure
ModBase 3D Structure for Q9NRG9
Purity/Purification
Affinity Purified
Form/Format
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Immunogen
The immunogen is a synthetic peptide directed towards the N-terminal region of Human AAAS
Preparation and Storage
For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent 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.
Related Product Information for
anti-AAAS antibody
This is a rabbit polyclonal antibody against AAAS. It was validated on Western Blot
Target Description: 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.
Product Categories/Family for anti-AAAS antibody
Polyclonal; Neuro
biology; Developmental Biology; Disease Related;
Applications Tested/Suitable for anti-AAAS antibody
Western Blot (WB)
Western Blot (WB) of anti-AAAS antibody
Host: Rabbit
Target Name: AAAS
Sample Type: OVCAR-3 Whole Cell lysates
Antibody Dilution: 1.0ug/ml

NCBI/Uniprot data below describe general gene information for AAAS. It may not necessarily be applicable to this product.
NCBI Accession #
NP_056480
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NCBI GenBank Nucleotide #
NM_015665.6
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UniProt Primary Accession #
Q9NRG9
[Other Products]
UniProt Related Accession #
Q9NRG9[Other Products]
NCBI Official Full Name
aladin isoform 1
NCBI Official Synonym Full Names
aladin WD repeat nucleoporin
NCBI Official Symbol
AAAS??[Similar Products]
NCBI Official Synonym Symbols
AAA; AAASb; GL003; ALADIN; ADRACALA; ADRACALIN
??[Similar Products]
NCBI Protein Information
aladin
UniProt Protein Name
Aladin
UniProt Synonym Protein Names
Adracalin
UniProt Gene Name
AAAS??[Similar Products]
UniProt Synonym Gene Names
ADRACALA??[Similar Products]
UniProt Entry Name
AAAS_HUMAN
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]
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: Membrane protein, integral; Nuclear envelope
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; gene expression; viral transcription; mitotic cell cycle; transmembrane transport
Disease: Achalasia-addisonianism-alacrima Syndrome
Research Articles on AAAS
1. AAAS mutations were identified in two families with hereditary spastic paraplegia.
Precautions
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