ELISA/assay

Human Ataxin 3 (ATXN3) ELISA Kit

MBS7208473

48-Strip-Wells

470 USD

ELISA Kit

Human Ataxin 3 (ATXN3) ELISA Kit

Ataxin 3 (ATXN3)

ataxin 3; Ataxin-3; ataxin-3; josephin; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; olivopontocerebellar ataxia 3; Machado-Joseph disease protein 1; spinocerebellar ataxia type 3 protein; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); ataxin 3; Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein

ATXN3

ATXN3; ATXN3; AT3; JOS; MJD; ATX3; MJD1; SCA3; ATX3; MJD; MJD1; SCA3

ATX3_HUMAN

Human

This assay has high sensitivity and excellent specificity for detection of ATXN3. No significant cross-reactivity or interference between ATXN3 and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between ATXN3 and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C.

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 0.1 ng/mL.

Intended Uses: This ATXN3 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human ATXN3. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

Neurobiology

Principle of the assay: ATXN3 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-ATXN3 antibody and an ATXN3-HRP conjugate. The assay sample and buffer are incubated together with ATXN3-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the ATXN3 concentration since ATXN3 from samples and ATXN3-HRP conjugate compete for the anti-ATXN3 antibody binding site. Since the number of sites is limited, as more sites are occupied by ATXN3 from the sample, fewer sites are left to bind ATXN3-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The ATXN3 concentration in each sample is interpolated from this standard curve.

152112974

ABS29269.1

20,633 Da

Protein Processing In Endoplasmic Reticulum Pathway (167325); Protein Processing In Endoplasmic Reticulum Pathway (167190)

Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Sep 2009]

ataxin-3: Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone- binding protein that regulates transcription. Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3); also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease. 3 isoforms of the human protein are produced by alternative splicing. Protein type: EC 3.4.19.12; Transcription regulation; Ubiquitin-specific protease; DNA repair, damage; Protease. Chromosomal Location of Human Ortholog: 14q21. Cellular Component: nucleoplasm; nuclear matrix; mitochondrial matrix; mitochondrial membrane; cytoplasm; nucleus; cytosol; nuclear inclusion body. Molecular Function: identical protein binding; protein binding; omega peptidase activity; ubiquitin protein ligase binding; ubiquitin-specific protease activity; ATPase binding. Biological Process: ubiquitin-dependent protein catabolic process; synaptic transmission; nervous system development; proteasomal ubiquitin-dependent protein catabolic process; transcription, DNA-dependent; regulation of transcription, DNA-dependent; nucleotide-excision repair; misfolded or incompletely synthesized protein catabolic process; intermediate filament cytoskeleton organization and biogenesis; microtubule cytoskeleton organization and biogenesis; actin cytoskeleton organization and biogenesis. Disease: Machado-joseph Disease

48-Strip-Wells

470 USD

96-Strip-Wells

675