脊髓小脑共济失调蛋白BEAN1抗体

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我们提供的抗体用于实验研究，而不用于诊临床断或治疗程序。
脊髓小脑共济失调蛋白BEAN1抗体Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
英文名称 Anti-BEAN1/SCA31
中文名称 脊髓小脑共济失调蛋白BEAN1抗体
别 名 BEAN; Bean1; BEAN1_HUMAN; Brain-expressed protein associating with Nedd4 homolog; Protein BEAN1; SCA31.
浓 度 1mg/1ml
规 格 0.2ml/200μg
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Horse
产品类型 一抗
研究领域 细胞生物 神经生物学
蛋白分子量 predicted molecular weight: 29kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human BEAN1 （10-70aa）
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
（石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 The protein encoded by this gene is one of several proteins that interact with NEDD4, a member of a family of ubiquitin-protein ligases. These proteins have PY motifs in common that bind to the WW domains of NEDD4. NEDD4 is developmentally regulated, and is highly expressed in embryonic tissues. Mutations in this gene （i.e., intronic insertions of >100 copies of pentanucleotide repeats including a （TGGAA）n sequence） are associated with spinocerebellar ataxia type 31. Alternatively spliced transcript variants encoding different isoforms have been
Subcellular Location : Membrane; Single-pass membrane protein （Potential）.
DISEASE : Defects in BEAN1 are the cause of spinocerebellar ataxia type 31 （SCA31） [MIM:117210]; also known as spinocerebellar ataxia 16q22-linked. A form of spinocerebellar ataxia, a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA31 belongs to the autosomal dominant cerebellar ataxias type III （ADCA III） which are characterized by pure cerebellar ataxia without additional signs.
Database links : UniProtKB/Swiss-Prot: Q3B7T3.2
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