Ataxin 1 Antikörper (AA 645-815)

Produktnummer ABIN7193320

Dieses Maus Monoklonal-Antikörper erkennt spezifisch Ataxin 1 in WB, ELISA und FACS. Er zeigt eine Reaktivität gegenüber Human und Maus.

Kurzübersicht für Ataxin 1 Antikörper (AA 645-815) (ABIN7193320)

Target: Ataxin 1 (ATXN1)

Reaktivität: Human, Maus

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Ataxin 1 Antikörper ist unkonjugiert

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

Klon: 2B8A2

Produktdetails anti-Ataxin 1 Antikörper

Bindungsspezifität: AA 645-815

Verwendungszweck: ATXN1 Antibody

Aufreinigung: Purified antibody

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

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

Hintergrund:

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.

Molekulargewicht: 86.9 kDa

UniProt: P54253

Pathways: Synaptic Membrane