Product Name
AAAS, Blocking Peptide
Full Product Name
AAAS Blocking Peptide (C-Term)
Product Synonym Names
Aladin; Adracalin; AAAS; ADRACALA
Product Gene Name
AAAS blocking peptide
[Similar Products]
Product Synonym Gene Name
ADRACALA[Similar Products]
AAAS peptide (MBS9230576) is used for blocking the activity of AAAS antibody (MBS9217015)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9NRG9
Specificity
The synthetic peptide sequence is selected from aa 415-425 of HUMAN AAAS
Form/Format
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
Cellular Location
Nucleus, nuclear pore complex
Tissue Location
Widely expressed. Particularly abundant expression is found in cerebellum, corpus callosum, adrenal gland, pituitary gland, gastrointestinal structures and fetal lung
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of AAAS blocking peptide 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
AAAS blocking peptide
Plays a role in the normal development of the peripheral and central nervous system.
NCBI/Uniprot data below describe general gene information for AAAS. It may not necessarily be applicable to this product.
NCBI Accession #
Q9NRG9.1
[Other Products]
UniProt Primary Accession #
Q9NRG9
[Other Products]
UniProt Secondary Accession #
Q5JB47; Q9NWI6; Q9UG19[Other Products]
UniProt Related Accession #
Q9NRG9[Other Products]
Molecular Weight
55,814 Da
NCBI Official Full Name
Aladin
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: centrosome; cytoplasm; membrane; nuclear envelope; nuclear membrane; nuclear pore; nucleoplasm; nucleus
Biological Process: mitotic nuclear envelope disassembly; mRNA export from nucleus; nucleocytoplasmic transport; protein sumoylation; RNA-mediated gene silencing; tRNA export from nucleus; viral reproduction; viral transcription
Disease: Achalasia-addisonianism-alacrima Syndrome
Research Articles on AAAS
1. Triple A syndrome with a novel indel mutation in the AAAS gene and delayed puberty.
Precautions
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