SACS Antibody [Alexa Fluor® 488]

• Reactivity: Hu
• Applications: IHC
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Alexa Fluor 488

SACS Antibody [Alexa Fluor® 488] Summary

• Immunogen: E. coli-derived recombinant human SACS
  Asn4402-Val4579
  Accession # Q9NZJ4
• Specificity: Detects human SACS in direct ELISAs.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep
• Purity Statement: Antigen Affinity-purified

Applications/Dilutions

• Dilutions:
  • Immunohistochemistry

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for SACS Antibody [Alexa Fluor® 488]

• ARSACS
• dnaJ homolog subfamily C member 29
• DNAJC29
• KIAA0730
• SACS
• Sacsin
• spastic ataxia of Charlevoix-Saguenay (sacsin)

Background

SACS (Spastic Ataxia of Charlevoix-Saguenay; also DNAJC29 and Sacsin) is a 520-540 kDa, novel cytoplasmic chaperone involved neuronal homeostasis. It is expressed in select cell types, including fibroblasts, skeletal muscle cells, cerebellar granule and Purkinje cells, and multiple CNS neuronal phenotypes. SACS is known to play a key role in protein folding, assisting in the adoption of a stable conformation, possibly through the use of ATP hydrolysis. It appears to collaborate with chaperone Hsp70 family proteins, and provide protection against polypeptides containing polyGlu repeats such as ataxin-1. These repeats tend to form annular structures, causing aggregates involving themselves and accompanying molecular partners. Human SACS is 4579 amino acids (aa) in length. It contains a N-terminal ubiquitin-like domain that associates with proteosomes (aa 9-84), three consecutive SRRs/Sacsin Supradomain Regions (aa 90-2900) that possess ATPase activity, a J-domain that intercats with other chaperones (aa 4306-4393), and a HEPN domain (aa 4451-4567) that binds GTP when SACS is dimerized. SACS contains three utilized Ser phosphorylation sites plus one acetylation site at Lys943. There is one alternate start site at Met751, and a 21 aa peptide that can substitute for either aa 730‑750, or aa 812-832. Over aa 4402-4579, human SACS shares 99% aa sequence identity with mouse SACS.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.