| 货号 | AF8014-SP |
| 别名 | ARSACS; dnaJ homolog subfamily C member 29; DNAJC29; KIAA0730; Sacsin; spastic ataxia of Charlevoix-Saguenay (sacsin) | 全称 | Spastic Ataxia of Charlevoix-Saguenay (Sacsin) |
| 反应种属 | Human |
| 应用 | Immunohistochemistry(5-15 µg/mL) |
| 目标/特异性 | Detects human SACS in direct ELISAs. |
| 使用方法 | Immunohistochemistry: 5-15 µg/mL |
| 来源 | Sterile PBS to a final concentration of 0.2 mg/mL. |
| 产品组分 |
| 供应商 | R&D Systems |
| Entrez Gene IDs | 26278 (Human); 50720 (Mouse); 305940 (Rat) |
| 纯化方式 | Antigen Affinity-purified |
| 免疫原 | E. coli-derived recombinant human SACS Asn4402-Val4579 Accession # Q9NZJ4 |
| 生物活性 | Human |
| 标记 | Unconjugated |
| 溶解方法 | Sterile PBS to a final concentration of 0.2 mg/mL. |
| 背景 | 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. |
| 运输条件 | Blue Ice |
| 存放说明 | 4℃ |
| 参考文献 |
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SACS in Human Brain. SACS was detected in immersion fixed paraffin-embedded sections of human brain (cerebellum) using Sheep Anti-Human SACS Antigen Affinity-purified Polyclonal Antibody (Catalog # AF8014) at 3 µg/mL overnight at 4 °C. Before incubation with the primary antibody, tissue was subjected to heat-induced epitope retrieval using Antigen Retrieval Reagent-Basic (Catalog # CTS013). Tissue was stained using the Anti-Sheep HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # CTS019) and counterstained with hematoxylin (blue). Specific staining was localized to Purkinje neurons. View our protocol for Chromogenic IHC Staining of Paraffin-embedded Tissue Sections. |
