Sacsin Antikörper (AA 3709-3909)

Produktnummer ABIN5693301

Produktdetails anti-Sacsin Antikörper

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

Bindungsspezifität: AA 3709-3909

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Sacsin Antikörper ist unkonjugiert

Applikation: ELISA, Immunohistochemistry (IHC), Western Blotting (WB)

Verwendungszweck: Anti-Sacsin Antibody Picoband®

Kreuzreaktivität (Details): No cross-reactivity with other proteins.

Produktmerkmale: Anti-Sacsin Antibody Picoband® (ABIN5693301). Tested in ELISA, IHC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Immunogen: E. coli-derived human Sacsin recombinant protein (Position: E3709-L3909).

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Western blot, 0.1-0.5 μg/mL
Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL
ELISA, 0.1-0.5 μg/mL
1. "Entrez Gene: SACS spastic ataxia of Charlevoix-Saguenay (sacsin)". 2. Engert JC, Doré C, Mercier J, Ge B, Bétard C, Rioux JD, Owen C, Bérubé P, Devon K, Birren B, Melan99on SB, Morgan K, Hudson TJ, Richter A (December 1999). "Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS): high-resolution physical and transcript map of the candidate region in chromosome region 13q11". Genomics. 62 (2): 156-64. 3. Parfitt DA, Michael GJ, Vermeulen EG, Prodromou NV, Webb TR, Gallo JM, Cheetham ME, Nicoll WS, Blatch GL, Chapple JP (May 2009). "The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1". Human Molecular Genetics. 18 (9): 1556-65

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Konzentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na₂HPO₄, 0.05 mg NaN₃.

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 -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Details zu Sacsin

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

Andere Bezeichnung: SACS (SACS Produkte)

Hintergrund:

Synonyms: Sacsin, DnaJ homolog subfamily C member 29, DNAJC29, SACS, KIAA0730

Tissue Specificity: Highly expressed in the central nervous system. Also found in skeletal muscle and at low levels in pancreas.

Background: Sacsin also known as DnaJ homolog subfamily C member 29 (DNAJC29) is a protein that in humans is encoded by the SACS gene. This gene consists of nine exons including a gigantic exon spanning more than 12.8k bp. It encodes the sacsin protein, which includes a UBQ region at the N-terminus, a HEPN domain at the C-terminus and a DnaJ region upstream of the HEPN domain. This modular protein is essential for normal mitochondrial network organization. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticityand peripheral neuropathy.

Molekulargewicht: 521 kDa

Gen-ID: 26278