Ataxin 1 Antikörper (AA 645-815)

Produktnummer ABIN7193320

Produktdetails anti-Ataxin 1 Antikörper

Target: Ataxin 1 (ATXN1)

Bindungsspezifität: AA 645-815

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Ataxin 1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Flow Cytometry (FACS)

Verwendungszweck: ATXN1 Antibody

Aufreinigung: Purified antibody

Immunogen: Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli.

Klon: 2B8A2

Isotyp: IgG1

Anwendungsinformationen

Applikationshinweise:

ELISA: 1/10000

FCM: 1/200 - 1/400

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Purified antibody in PBS with 0.05 % sodium azide.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Details zu Ataxin 1

Target: Ataxin 1 (ATXN1)

Andere Bezeichnung: ATXN1 (ATXN1 Produkte)

Synonyme: ATX1 antikoerper, D6S504E antikoerper, SCA1 antikoerper, ATXN1 antikoerper, ataxin 1b antikoerper, atxn1 antikoerper, 2900016G23Rik antikoerper, Atx1 antikoerper, C85907 antikoerper, ENSMUSG00000074917 antikoerper, Gm10786 antikoerper, Sca1 antikoerper, CG4547 antikoerper, Dmel\\CG4547 antikoerper, dAtx-1 antikoerper, dAtx1 antikoerper, sca1 antikoerper, ataxin 1 antikoerper, ataxin 1b antikoerper, Ataxin 1 antikoerper, ATXN1 antikoerper, atxn1b antikoerper, Atxn1 antikoerper, Atx-1 antikoerper

Hintergrund:

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. 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 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames.

Aliases: ATX1, SCA1, D6S504E

Molekulargewicht: 86.9kDa

Gen-ID: 6310

UniProt: P54253

Pathways: Synaptic Membrane