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Key Documents

AV54296

Sigma-Aldrich

Anti-GLA antibody produced in rabbit

affinity isolated antibody

Synonym(s):

Anti-α-Galactosidase, Anti-GALA

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

Quality Level

100

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

45 kDa

species reactivity

rat, mouse, guinea pig, human, dog

concentration

0.5 mg - 1 mg/mL

technique(s)

western blot: suitable

NCBI accession no.

NP_000160

UniProt accession no.

P06280

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... GLA(2717)

Immunogen

Synthetic peptide directed towards the N terminal region of human GLA

Application

Anti-GLA antibody produced in rabbit is suitable for western blotting at a concentration of 1μg/mL.

Biochem/physiol Actions

GLA gene encodes a homodimeric glycoprotein that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins. It also plays a role in catalysing hydrolysis of melibiose into galactose and glucose. Mutation in GLA gene results in fabry disease, a rare lysosomal storage disorder associated with failure to catabolize α-D-galactosyl glycolipid moieties.

Sequence

Synthetic peptide located within the following region: PQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTF

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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D P Germain et al.
Biochemical and biophysical research communications, 257(3), 708-713 (1999-04-20)
Fabry disease (FD) (angiokeratoma corporis diffusum) is an X-linked inborn error of glycosphingolipid metabolism caused by defects in the lysosomal alpha-galactosidase A gene (GLA). The enzymatic defect leads to the systemic accumulation of neutral glycosphingolipids with terminal alpha-galactosyl moieties. Clinically

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