Mouse Anti-AAAS Recombinant Antibody (V2-12483) (CBMAB-0973-CN)

This product is a mouse antibody that recognizes AAAS of human. The antibody 3E9 can be used for immunoassay techniques such as: WB, ELISA, IF, IHC-P, IP.
See all AAAS antibodies

Published Data

Mouse Anti-AAAS Recombinant Antibody (3E9) (CBMAB-0973-CN) in IF

ALADIN localizes around the meiotic spindle during mouse oocyte maturation. (A) Wild-type oocytes at various stages of meiotic progression were fixed and immunostained for ALADIN and α-tubulin and then costained with DAPI. Representative wide-field fluorescence images from a single z-plane are shown. Scale bar = 50 μm. ...View More

Carvalhal, S., Stevense, M., Koehler, K., Naumann, R., Huebner, A., Jessberger, R., & Griffis, E. R. (2017). ALADIN is required for the production of fertile mouse oocytes. Molecular biology of the cell, 28(19), 2470-2478.

Datasheet

Summary

Host Animal

Mouse

Specificity

Human, Mouse, Rat

Clone

V2-12483

Antibody Isotype

IgG2a

Application

WB, ELISA, IF, IHC-P, IP

Basic Information

Immunogen

Raised against recombinant Aladin of human origin

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG2a

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:100-1:1,000
IHC	1:50-1:500
ELISA	1:30-1:3,000
IF(ICC)	1:50-1:500
IP	1-2 µl per 100-500 µg of total protein (1 ml of cell lysate)

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS, 0.1% gelatin

Preservative

< 0.1% sodium azide

Concentration

0.1 mg/ml

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name

achalasia, adrenocortical insufficiency, alacrimia (Allgrove, triple-A)

Introduction

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. This gene plays a role in the normal development of the peripheral and central nervous system.

Entrez Gene ID

Human	8086
Mouse	223921
Rat	300259

UniProt ID

Human	Q9NRG9
Mouse	P58742

Alternative Names

AAA; AAASb; GL003; ALADIN; ADRACALA; ADRACALIN

Function

Plays a role in the normal development of the peripheral and central nervous system (PubMed:11062474. Required for the correct localization of aurora kinase AURKA and the microtubule minus end-binding protein NUMA1 as well as a subset of AURKA targets which ensures proper spindle formation and timely chromosome alignment.

Biological Process

Fertilization
Intracellular transport of virus
Learning
Microtubule bundle formation
Mitotic spindle assembly
mRNA export from nucleus
Nucleocytoplasmic transport
Protein sumoylation
Regulation of cellular response to heat
Regulation of gene silencing by miRNA
Regulation of glycolytic process
Regulation of nucleocytoplasmic transport
tRNA export from nucleus
Viral life cycle
Viral process
Viral transcription

Cellular Location

Nuclear pore complex; Nucleus envelope; Spindle pole. In metaphase cells localizes within the spindle with some accumulation around spindle poles, with the highest concentration between the centrosome and metaphase plate. The localization to the spindle is microtubule-mediated.

Involvement in disease

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.

References

Jayant, S. S., Gupta, R., Agrawal, K., Das, L., Dutta, P., & Bhansali, A. (2021). Triple A (Allgrove) syndrome due to AAAS gene mutation with a rare association of amyotrophy. Hormones, 20(1), 197-205.

Vezzoli, V., Duminuco, P., Pogliaghi, G., Saccone, M., Cangiano, B., Rosatelli, M. C., ... & Bonomi, M. (2020). Two novel truncating variants of the AAAS gene causative of the triple A syndrome. Journal of endocrinological investigation, 1-10.

Bouliari, A., Lu, X., Persky, R. W., & Stratakis, C. A. (2019). Triple A syndrome: Two siblings with a novel mutation in the AAAS gene. Hormones, 18(1), 109-112.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Associated Products

Related Products

Mouse Anti-AAAS Recombinant Antibody (V2-178851)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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