AAA; AAASb; GL003; ALADIN; ADRACALA; ADRACALIN

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Antigen affinity purification

1mg/mL (lot specific)

Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Manufactured in an ISO 9001:2015 Certified Laboratory.

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.

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.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:200-1:1000
IHC: 1:50-1:200

Western Blot analysis of 293T, hela and PC3 cell using AAAS Polyclonal Antibody at dilution of 1:400

Immunohistochemistry of paraffin-embedded Human thyroid cancer using AAAS Polyclonal Antibody at dilution of 1:20

Immunohistochemistry of paraffin-embedded Human brain using AAAS Polyclonal Antibody at dilution of 1:20

55,814 Da

achalasia, adrenocortical insufficiency, alacrimia

aladin; Allgrove, triple-A

Aladin

ADRACALA??[Similar Products]

AAAS_HUMAN

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]

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; viral infectious cycle; learning; glucose transport; protein transport; fertilization; regulation of nucleocytoplasmic transport; hexose transport; carbohydrate metabolic process; viral transcription; gene expression; mitotic cell cycle; transmembrane transport

Disease: Achalasia-addisonianism-alacrima Syndrome

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