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D0319

Sigma-Aldrich

Anti-Dorfin (N-terminal) antibody produced in rabbit

~1.5 mg/mL, affinity isolated antibody, buffered aqueous solution

Synonym(s):

Anti-Double ring-finger protein, Anti-Protein p38 interacting with transcription factor Sp1, Anti-RING finger protein 19, Anti-RNF19, Anti-p38 protein

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~105 kDa

species reactivity

rat (predicted), human, mouse

concentration

~1.5 mg/mL

technique(s)

western blot: 1-2 μg/mL using HEK-293T cells expressing human Dorfin

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

Gene Information

human ... RNF19A(25897)
mouse ... Rnf19a(30945)

General description

Dorfin is an E3 ubiquitin ligase that contains a RING finger/IBR (in between-RING-finger) domain at its N-terminus. Dorfin resides in the perinuclear region and forms aggresome-like structures, that contain misfolded ubiquitylated proteins.

Immunogen

synthetic peptide corresponding to amino acids 1-19 of human dorfin, conjugated to KLH. This sequence is identical in mouse dorfin and highly conserved (two amino acid substitutions) in rat dorfin.

Application

Anti-Dorfin (N-terminal) antibody produced in rabbit is suitable for immunoblotting at a working concentration of 1-2 μg/mL using HEK-293T cells expressing human Dorfin.

Biochem/physiol Actions

Dorfin mediates E3 ubiquitin ligase activity. It interacts with human E2s UbcH7 and UbcH8. The C-terminus of Dorfin can recognize mutant SOD1 proteins, which cause familial ALS (FALS). Dorfin binds and ubiquitinylates various SOD1 mutants derived from FALS and enhances their degradation, but has no effect on the stability of wild-type SOD1. Over expression of this protein protects neuronal cells against the toxic effect of mutant SOD1 and reduces SOD1 inclusions. It has been localized to aggresomes and ubiquitin positive inclusions in several neurodegenerative diseases including: PD, dementia with Lewy bodies, multiple system atrophy and ALS.

Physical form

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

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 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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Nozomi Hishikawa et al.
The American journal of pathology, 163(2), 609-619 (2003-07-24)
In many neurodegenerative diseases, the cytopathological hallmark is the presence of ubiquitylated inclusions consisting of insoluble protein aggregates. Lewy bodies in Parkinson's disease and dementia with Lewy bodies disease, glial cell inclusions in multiple system atrophy, and hyaline inclusions in
Jun-Ichi Niwa et al.
The Journal of biological chemistry, 277(39), 36793-36798 (2002-07-30)
Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder resulting from the degeneration of motor neurons in the cerebral cortex, brainstem, and spinal cord. The cytopathological hallmark in the remaining motor neurons of ALS is the presence of ubiquitylated inclusions
J Niwa et al.
Biochemical and biophysical research communications, 281(3), 706-713 (2001-03-10)
We cloned a novel gene, Dorfin (double ring-finger protein), from human spinal cord. The Dorfin mRNA transcript was 4.4 kb and expressed ubiquitously in many organs as well as in the central nervous system, including the spinal cord. Dorfin encoded
Takashi Ito et al.
The Journal of biological chemistry, 278(31), 29106-29114 (2003-05-17)
Parkinson's disease (PD) is a neurodegenerative disease characterized by loss of nigra dopaminergic neurons. Lewy bodies (LBs) are a characteristic neuronal inclusion in PD brains. In this study, we report that Dorfin, a RING finger-type ubiquityl ligase for mutant superoxide

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