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Sacsin 抗体 (AA 3709-3909)

SACS 适用: 人, 小鼠, 大鼠 ELISA, IHC, WB 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN5693301
发货至: 中国
  • 抗原 See all Sacsin (SACS) 抗体
    Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))
    抗原表位
    • 5
    • 1
    • 1
    • 1
    AA 3709-3909
    适用
    人, 小鼠, 大鼠
    宿主
    • 9
    • 1
    克隆类型
    • 10
    多克隆
    标记
    • 6
    • 2
    • 1
    • 1
    This Sacsin antibody is un-conjugated
    应用范围
    • 6
    • 3
    • 2
    • 2
    • 1
    • 1
    ELISA, Immunohistochemistry (IHC), Western Blotting (WB)
    原理
    Anti-Sacsin Antibody Picoband®
    交叉反应 (详细)
    No cross-reactivity with other proteins.
    产品特性
    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.
    免疫原
    E. coli-derived human Sacsin recombinant protein (Position: E3709-L3909).
    亚型
    IgG
    Top Product
    Discover our top product SACS Primary Antibody
  • 应用备注
    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
    限制
    仅限研究用
  • 状态
    Lyophilized
    溶解方式
    Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
    浓度
    500 μg/mL
    缓冲液
    Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg NaN3.
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    储存条件
    4 °C,-20 °C
    储存方法
    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.
  • 抗原
    Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))
    别名
    SACS (SACS 产品)
    背景

    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.

    分子量
    521 kDa
    基因ID
    26278
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