产品类别

联系我们

电话:025-68037686

地址:江苏生命科技创新园F6幢1层

订购:nanjing03@biogot.com

服务:biorase01@biogot.com

合作:lvyun@biogot.com

支持:may@biogot.com

ATXN1 monoclonal antibody

货号: MB22690

价格：￥ 1200

货期：现货（1-2个工作日）

25ug 50ug 100ug 200ug

抗体定制服务咨询

说明书 MSDS SHIP

详细信息检测数据实验指南文献

No. :MB22690

Product Name :ATXN1 monoclonal antibody

Swiss-Prot :P54253

Host :Mouse

Reactivity :Human,Mouse

Applications :WB,FCM

Application_all :WB:1/500 - 1/2000 FC:1/200 - 1/400

Background :The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the `pure' cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames.</p>

Product :Purified antibody in PBS with 0.05% sodium azide

Purification&Purity :The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity :Mouse IgG1

BiowMW :86.9kDa

Note :For research use only, not for use in diagnostic procedure.

Alternative Name :ATX1; SCA1; D6S504E

Immunogen :Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli.

Conjugate :Unconjugated

Modification :Unmodification

Western blot analysis using ATXN1 mouse mAb against COS7 (1), NIH/3T3 (2), and HL-60 (3) cell lysate.

免疫沉淀（IP）

免疫组织化学（IHC）

免疫荧光（IF）