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HPA030200

Sigma-Aldrich

Anti-DST antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

Synonym(s):

Anti-BP240, Anti-BPA, Anti-BPAG1, Anti-CATX-15, Anti-FLJ13425, Anti-FLJ21489, Anti-FLJ30627, Anti-FLJ32235, Anti-KIAA0728, Anti-MACF2, Anti-dystonin

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About This Item

UNSPSC Code:
12352203
Human Protein Atlas Number:
NACRES:
NA.41

biological source

rabbit

Quality Level

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

product line

Prestige Antibodies® Powered by Atlas Antibodies

form

buffered aqueous glycerol solution

species reactivity

human

technique(s)

immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200

immunogen sequence

KPRCQNVAEDMDIQLCASILNENSDENENINTMILLDKMHSCSSLEKQQRVNVVQLASPSENNLVTEKSNLPEYTTEIAGKSKENLLNHE

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... DST(667)

General description

DST gene encodes dystonin, a cytoskeletal linker protein. Dystonin proteins are present as several isoforms in neural and muscle cells. DST gene is located on the human chromosome 6p12.1. Dystonin protein encompasses plakin and spectrin domains.

Immunogen

dystonin recombinant protein epitope signature tag (PrEST)

Application

Anti-DST antibody produced in rabbit is suitable for human dystonin detection in live cell microscopy.

Biochem/physiol Actions

DST integrates microtubules, actin and intermediate filaments and is essential for cytoskeleton network in neuronal cells. A frame shift mutation in the DST gene affects sensory and autonomous nervous system, leading to hereditary sensory autonomic neuropathy type VI. The incidence of nonsense mutation in the DST gene is implicated in the skin disorder.

Features and Benefits

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

Linkage

Corresponding Antigen APREST77625

Physical form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.

Legal Information

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

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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WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Information

新产品

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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Cloning and characterization of the neural isoforms of human dystonin
Brown A, et al.
Genomics, 29(3), 777-780 (1995)
A homozygous nonsense mutation within the dystonin gene coding for the coiled-coil domain of the epithelial isoform of BPAG1 underlies a new subtype of autosomal recessive epidermolysis bullosa simplex
Groves RW, et al.
The Journal of Investigative Dermatology, 130(6), 1551-1557 (2010)
Structural analysis of the plakin domain of bullous pemphigoid antigen1 (BPAG1) suggests that plakins are members of the spectrin superfamily
Jefferson JJ, et al.
Journal of Molecular Biology, 366(1), 244-257 (2007)
Human homolog of a mouse sequence from the dystonia musculorum locus is on chromosome 6p12
Brown A, et al.
Mammalian Genome, 5(7), 434-437 (1994)
Hereditary sensory autonomic neuropathy caused by a mutation in dystonin
Edvardson S, et al.
Annals of Neurology, 71(4), 569-572 (2012)

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