Ataxin 1 Antikörper (N-Term)

Produktnummer ABIN5514519

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch Ataxin 1 in WB. Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für Ataxin 1 Antikörper (N-Term) (ABIN5514519)

Target: Ataxin 1 (ATXN1)

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Ataxin 1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktdetails anti-Ataxin 1 Antikörper

Bindungsspezifität: N-Term

Sequenz: TAWLPGNPGG RGHGGGRHGP AGTSVELGLQ QGIGLHKALS TGLDYSPPSA

Aufreinigung: Affinity purified

Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human ATXN1

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Konservierungsmittel: Sodium azide

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

Lagerung: -20 °C

Informationen zur Lagerung: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent 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 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.

Alias Symbols: ATX1, SCA1, D6S504E

Protein Size: 815

Gen-ID: 6310

NCBI Accession: NM_000332, NP_000323

UniProt: P54253

Pathways: Synaptic Membrane