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Ataxin 1 Recombinant Rabbit mAb (KU)

货号: BS46701

价格：￥ 1200

货期：现货（1-2个工作日）

25ul 50ul 100ul

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说明书 MSDS SHIP

详细信息检测数据实验指南文献

No. :BS46701

Product Name :Ataxin 1 Recombinant Rabbit mAb (KU)

Swiss-Prot :P54253

Host :Rabbit

Reactivity :Human, Mouse, Rat

Applications :WB,IP

Application_all :WB,1:500 - 1:2000|IP,1:50 - 1:200

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). At least two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2016]

Product :1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Purification&Purity :The antibody was affinity-purified from rabbit antiserum 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 :Polyclonal Antibodies

BiowMW :108kDa

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

Alternative Name :NLRX1;CLR11.3;DLNB26;NOD26;NOD5;NOD9

Immunogen :Recombinant fusion protein of human NLRX1(NP_001269073.1).

Conjugate :Unconjugated

Modification :Unmodification

Western blot analysis of extracts of various cell lines, using NLRX1 antibody at 1:3000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit .
Exposure time: 10s.