AGA Antibody

Code: CSB-PA213113

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Size:

50μl100μl

US$166

Quantity:

Species Reactivity: Human,Mouse,Rat

Raised in: Rabbit

Application: ELISA,WB

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Product Details
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Target Background

Product Details

Uniprot NO.

P20933

Target Names

AGA

Alternative Names

Aga antibody; AGU antibody; Aspartylglucosaminidase antibody; Aspartylglucosylamine deaspartylase antibody; Aspartylglycosaminuria antibody; ASPG_HUMAN antibody; ASRG antibody; GA antibody; Glycosylasparaginase antibody; Glycosylasparaginase beta chain antibody; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase antibody; N4 (N acetyl beta glucosaminyl) L asparagine amidase antibody; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase antibody

Raised in

Rabbit

Species Reactivity

Human,Mouse,Rat

Immunogen

Fusion protein of Human AGA

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Isotype

IgG

Purification Method

Antigen affinity purification

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Form

Liquid
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Tested Applications

ELISA,WB

Protocols

ELISA Protocol Western Blotting(WB) Protocol

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Usage

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

Datasheet & COA

Antibody FAQs

Images

Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Raji cells, Primary antibody: CSB-PA213113(AGA Antibody) at dilution 1/125, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds

Customer Reviews and Q&A

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Target Background

Function(From Uniprot)

Cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins.

Gene References into Functions

1.8A resolution crystal structure of mature G172D mutant of a model missense GA corresponding to a Canadian aspartylglucosaminuria allele; studied the effect of its single amino acid change on substrate processing PMID:28457719
We show that gene-silenced cells show specifically reduced AGA activity and store globotriaosylceramide. In gene-silenced cells, release of the neurotransmitter acetylcholine is significantly reduced, demonstrating that this model may be used to study specific neuronal functions such as neurotransmitter release in Fabry disease PMID:27471012
study reports 2 novel aspartylglucosaminidase gene mutations, one in Qatari twins with an early, perinatal presentation not previously described for aspartylglucosaminuria and the other in 3 Turkish children with newly diagnosed aspartylglucosaminuria and a more classical disease course PMID:23271757
[review] Natural killer (NK) cell tumors, subtypes of myeloid leukemias and T-cell lymphomas respond to ASNase; ovarian carcinomas and other solid tumors have been proposed as additional targets for ASNase, with a potential role for glutaminase. activity. PMID:21854356
Molecular mechanism for the autoproteolytic activation of aspartylglucosaminidase. PMID:14616088
A new point mutation, c.44T>G, found in a Finnish compound heterozygote causes a L15R AA substitution in the signal sequence of the AGA enzyme, affecting AGA translocation by altering a critical hydrophobic core structure in the signal sequence. PMID:15365992
aspartylglucosaminidase may have a role in development of congenital disorders of glycosylation type I PMID:16435229
The amino acid substitutions in aspartylglucosaminidase responsible for aspartylglucosaminuria were classified and divided in three groups. PMID:18992224
Increased AGA plasma activity, although a consistent finding in congenital disorders of glycosylation patients, is not specific to this group of disorders. PMID:19100247