Thermo Fisher Scientific ATXN1 Monoclonal Antibody (4C5)

Applications

Tested Dilution

Publications

Western Blot (WB)

1-5 µg/mL

-

ELISA (ELISA)

10 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG2b, kappa

Class

Monoclonal

Type

Antibody

Clone

4C5

Immunogen

ATXN1 (NP_000323, 576 a.a. approximately 675 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:Ataxin 1,uniProtId:P54253-1,ncbiNodeId:9606,antigenRange:576-675,antigenLength:815,antigenImageFileName:H00006310-M02_Ataxin_1_P54253-1_House_mouse.svg,antigenImageFileNamePDP:H00006310-M02_Ataxin_1_P54253-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

See Label

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.4

Contains

no preservative

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Sequence of this protein is as follows: KGSIIQLANG ELKKVEDLKT EDFIQSAEIS NDLKIDSSTV ERIEDSHSPG VAVIQFAVGE HRAQVSVEVL VEYPFFVFGQ GWSSCCPERT SQLFDLPCSK

Target Information

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. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the `pure` cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 41-81 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). At least two transcript variants encoding the same protein have been found for this gene.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.