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Quantity: 100 ul                                                                                             Application: WB

Predicted I Observed M.W.: 95 kDa                                                            Uniprot ID: O15265

Background:

The autosomal dominant cerebellar ataxias (ADCA) are heterogeneous 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. This locus has been mapped to chromosome 3, and it has been determined that the diseased allele associated with spinocerebellar ataxia-7 contains 38-130 CAG repeats (near the N-terminus), compared to 7-17 in the normal allele. Ataxin-7 (ATXN7) is a component of the SPT3/TAF9/GCN5 acetyltransferase (STAGA) and TBP-free TAF-containing (TFTC) chromatin remodeling complexes, and it thus plays a role in transcriptional regulation. Alternative splicing results in multiple transcript variants [provided by RefSeq].

Other Names:

Ataxin-7, Spinocerebellar ataxia type 7 protein, SCA7, ADCAII, OPCA3

Source and Purity:

Rabbit polyclonal antibodies were produced by immunizing animals with a GST-fusion protein containing the C-terminal region of human ATXN7. Antibodies were purified by affinity purification using immunogen. 

Storage Buffer and Condition:

Supplied in 1 x PBS (pH 7.4), 100 ug/ml BSA, 40% Glycerol, 0.01% NaN3. Store at -20 °C. Stable for 6 months from date of receipt.

Species Specificity:

Human

Tested Applications: 

WB: 1:1,000-1:3,000 (detect endogenous protein*)

*: The apparent protein size on WB may be different from the calculated M.W. due to modifications.

Product Data: