Human Ataxin 2 ELISA Kit | ATXN2 elisa kit

Human Ataxin 2 ELISA Kit

Cat. Num. AAA726216

• Gene Names: ATXN2; ATX2; SCA2; ASL13; TNRC13
• Reactivity: Human
• Synonyms: Ataxin 2; Human Ataxin 2 ELISA Kit; ATXN2 elisa kit

• Reactivity: Human

• Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
• Assay Type: Competitive
• Detection Range: 1.0-25ng/mL
• Sensitivity: 0.1ng/mL
• Intended Uses: This ATXN2 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human ATXN2. This ELISA kit for research use only, not for therapeutic applications!
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for ATXN2 elisa kit

Principle of the Assay: ATXN2 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-ATXN2 antibody and an ATXN2-HRP conjugate. The assay sample and buffer are incubated together with ATXN2-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 ATXN2 concentration since ATXN2 from samples and ATXN2-HRP conjugate compete for the anti-ATXN2 antibody binding site. Since the number of sites is limited, as more sites are occupied by ATXN2 from the sample, fewer sites are left to bind ATXN2-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The ATXN2 concentration in each sample is interpolated from this standard curve.

Product Categories/Family for ATXN2 elisa kit

Neurobiology

NCBI and Uniprot Product Information

• NCBI GI #: 171543895
• NCBI GeneID: 6311
• NCBI Accession #: NP_002964.3
• NCBI GenBank Nucleotide #: NM_002973.3
• Molecular Weight: 132,884 Da
• NCBI Official Full Name: ataxin-2
• NCBI Official Synonym Full Names: ataxin 2
• NCBI Official Symbol: ATXN2
• NCBI Official Synonym Symbols: ATX2; SCA2; ASL13; TNRC13
• NCBI Protein Information: ataxin-2; spinocerebellar ataxia type 2 protein; trinucleotide repeat-containing gene 13 protein
• UniProt Protein Name: Ataxin-2
• Protein Family: Ataxin
• UniProt Gene Name: ATXN2
• UniProt Synonym Gene Names: ATX2; SCA2; TNRC13
• UniProt Entry Name: ATX2_HUMAN

NCBI Description

The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. Defects in this gene are the cause of spinocerebellar ataxia type 2 (SCA2). SCA2 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. SCA2 is caused by expansion of a CAG repeat in the coding region of this gene. This locus has been mapped to chromosome 12, and it has been determined that the diseased allele contains 37-50 CAG repeats, compared to 17-29 in the normal allele. Longer expansions result in earlier onset of the disease. Alternatively spliced transcript variants encoding different isoforms have been identified but their full length sequence has not been determined. [provided by RefSeq, Jan 2010]

Uniprot Description

ataxin-2: Involved in EGFR trafficking, acting as negative regulator of endocytic EGFR internalization at the plasma membrane. Defects in ATXN2 are the cause of spinocerebellar ataxia type 2 (SCA2); also known as olivopontocerebellar atrophy II (OPCA II or OPCA2). 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 cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA2 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. SCA2 is characterized by hyporeflexia, myoclonus and action tremor and dopamine-responsive parkinsonism. SCA2 is caused by expansion of a CAG repeat resulting in about 36 to 52 repeats in some patients. Longer expansions result in earlier the expansion, onset of the disease. Defects in ATXN2 are a cause of susceptibility to amyotrophic lateral sclerosis type 13 (ALS13). It is a neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. An increased risk for developing amyotrophic lateral sclerosis is seems to be conferred by CAG repeat intermediate expansions greater than 23 but below the threshold for developing spinocerebellar ataxia. Belongs to the ataxin-2 family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA-binding; Translation

Chromosomal Location of Human Ortholog: 12q24.1

Cellular Component: nucleoplasm; polysome; Golgi apparatus; membrane; stress granule; perinuclear region of cytoplasm; cytoplasm; trans-Golgi network; ribonucleoprotein complex

Molecular Function: protein C-terminus binding; protein binding; RNA binding; epidermal growth factor receptor binding

Biological Process: regulation of translation; stress granule assembly; negative regulation of multicellular organism growth; RNA metabolic process; neuromuscular process; cerebellar Purkinje cell differentiation; homeostasis of number of cells; cytoplasmic mRNA processing body assembly; neurite morphogenesis; RNA transport; negative regulation of receptor internalization

Disease: Parkinson Disease, Late-onset; Spinocerebellar Ataxia 2