Mouse monoclonal antibody raised against partial recombinant human ATXN1.
Immunogen:
Recombinant protein corresponding to amino acids 645-815 of human ATXN1.
Host:
Mouse
Theoretical MW (kDa):
86.9
Reactivity:
Human
Form:
Liquid
Isotype:
IgG1
Recommend Usage:
ELISA (1:10000) Flow Cytometry (1:200-1:400) Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (1:200-1:1000) Western Blot (1:500-1:2000) The optimal working dilution should be determined by the end user.
Storage Buffer:
In PBS (0.05% sodium azide).
Storage Instruction:
Store at 4°C. For long term storage store at -20°C. Aliquot to avoid repeated freezing and thawing.
Note:
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Western Blot analysis of Lane 1: HEK293 and Lane 2: ATXN1-hIgGFc transfected HEK293 cell lysates with ATXN1 monoclonal antibody, clone 4C7B11 (Cat # MAB21432).
Immunohistochemical staining (Formalin-fixed paraffin-embedded sections) of human endometrium cancer with ATXN1 monoclonal antibody, clone 4C7B11 (Cat # MAB21432).
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 41-81 CAG repeats, compared to 6-39 in the normal allele. At least two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq