脊髓小脑共济失调蛋白叠贰础狈1抗体
规格:1尘驳/1尘濒
英文名: BEAN1
别名: BEAN; SCA31; Bean1; BEAN1_HUMAN; Brain-expressed protein associating with Nedd4 homolog; Protein BEAN1; SCA31.
分子量: 29kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:笔辞濒测肠濒辞苍补濒
亚型:滨驳骋
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human BEAN1 (1
交叉反应:Human, Mouse, Rat, Dog, Cow, Horse,
细胞定位:细胞膜
脊髓小脑共济失调蛋白叠贰础狈1抗体产物介绍:background: 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 found for this gene. [provided by RefSeq, May 2010]. Subunit: Interacts with NEDD4脊髓小脑共济失调蛋白叠贰础狈1抗体 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, 脊髓小脑共济失调蛋白叠贰础狈1抗体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. Gene ID: 146227 Database links: Entrez Gene: 146227 Human Omim: 612051 Human SwissProt: Q3B7T3 Human Unigene: 97805 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
脊髓小脑共济失调蛋白叠贰础狈1抗体产物应用: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.
研究领域:细胞生物 神经生物学
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid