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Key Documents

SAB1407430

Sigma-Aldrich

Anti-EXOSC3 antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

Synonym(s):

CGI-102, MGC15120, MGC723, RP11-3J10.8, RRP40, Rrp40p, bA3J10.7, hRrp40p, p10

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

Quality Level

100

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~29.6 kDa

species reactivity

human

technique(s)

indirect immunofluorescence: suitable
western blot: 1 μg/mL

NCBI accession no.

NM_016042.2

UniProt accession no.

Q9NQT5

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... EXOSC3(51010)

Immunogen

EXOSC3 (NP_057126.2, 1 a.a. ~ 275 a.a) full-length human protein.

Sequence
MAEPASVAAESLAGSRARAARTVLGQVVLPGEELLLPEQEDAEGPGGAVERPLSLNARACSRVRVVCGPGLRRCGDRLLVTKCGRLRHKEPGSGSGGGVYWVDSQQKRYVPVKGDHVIGIVTAKSGDIFKVDVGGSEPASLSYLSFEGATKRNRPNVQVGDLIYGQFVVANKDMEPEMVCIDSCGRANGMGVIGQDGLLFKVTLGLIRKLLAPDCEIIQEVGKLYPLEIVFGMNGRIWVKAKTIQQTLILANILEACEHMTSDQRKQIFSRLAES

Physical form

Solution in phosphate buffered saline, pH 7.4

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

10 - Combustible liquids

wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificates of Analysis (COA)

Lot/Batch Number
12144
10358

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Ayelet Halevy et al.
Journal of neurology, 261(11), 2165-2169 (2014-08-26)
We describe two pairs of siblings from a consanguineous family manifesting autosomal recessive hereditary spastic paraplegia caused by a novel mutation in the EXOSC3 gene, previously reported in pontocerebellar hypoplasia type 1. Clinical findings included delayed motor milestones, early-onset spastic
Margaret P Adam et al.
GeneReviews(?), 2014 Aug 21 (2014-08-22)
The diagnosis is suspected in infants and children with characteristic clinical findings (including evidence of anterior horn cell disease) and characteristic neuroradiologic findings (hypoplasia and/or atrophy of the cerebellum and pons in varying degrees; equal involvement of the cerebellar vermis
Veerle Rc Eggens et al.
Orphanet journal of rare diseases, 9, 23-23 (2014-02-15)
Pontocerebellar hypoplasia (PCH) represents a group of neurodegenerative disorders with prenatal onset. Eight subtypes have been described thus far (PCH1-8) based on clinical and genetic features. Common characteristics include hypoplasia and atrophy of the cerebellum, variable pontine atrophy, and severe
J Xu et al.
Cell death and differentiation, 21(8), 1229-1239 (2014-04-29)
Macrophages can be activated and regulated by high-mobility group box 1 (HMGB1), a highly conserved nuclear protein. Inflammatory functions of HMGB1 are mediated by binding to cell surface receptors, including the receptor for advanced glycation end products (RAGE), Toll-like receptor

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