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AAAS, Blocking Peptide

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AAAS, Blocking Peptide


AAAS, Blocking Peptide  的詳細介紹
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]
Antibody/Peptide Pairs
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.
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OMIM
231550
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.
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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 GI #
20137527
NCBI GeneID
8086
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
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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
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: 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.
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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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