Sacsin Antikörper (C-Term)

Produktnummer ABIN5673657

Dieser Kaninchen Polyklonal Antikörper erkennt spezifisch Sacsin in WB. Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für Sacsin Antikörper (C-Term) (ABIN5673657)

Target: Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Sacsin Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktdetails anti-Sacsin Antikörper

Bindungsspezifität: C-Term

Sequenz: VVEQAWKLPE SERKKIIRRL YLKWHPDKNP ENHDIANEVF KHLQNEINRL

Aufreinigung: Affinity purified

Immunogen: The immunogen is a synthetic peptide directed towards the C terminal region of human SACS

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 Sacsin

Target: Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))

Andere Bezeichnung: SACS

Hintergrund: This gene encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. This gene includes a very large exon spanning more than 12.8 kb. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins". A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants.

Alias Symbols: SPAX6, ARSACS, DNAJC29, PPP1R138

Protein Size: 309

Gen-ID: 26278

NCBI Accession: NM_014363, NP_055178

UniProt: Q7Z6W7