Specifications
- HOST SPECIES: Rabbit
- SPECIES REACTIVITY: Human
- HOMOLOGY: Predicted species reactivity based on immunogen sequence: Mouse
- IMMUNOGEN: This ATXN7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 354-381 amino acids from the Central region of human ATXN7.
- CONJUGATE: Unconjugated
- TESTED APPLICATIONS: WB
- APPLICATION NOTE: For WB starting dilution is: 1:1000
- PREDICTED MOLECULAR WEIGHT: 95 kDa
Properties
- PURIFICATION: This antibody is purified through a protein A column, followed by peptide affinity purification.
- CLONALITY: Polyclonal
- ISOTYPE: Rabbit Ig
- PHYSICAL STATE: Liquid
- BUFFER: Supplied in PBS with 0.09% (W/V) sodium azide.
- CONCENTRATION: batch dependent
- STORAGE CONDITIONS: Store at 4°C for three months and -20°C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Additional Info
- NCBI OFFICIAL SYMBOL: ATXN7
- ADDITIONAL NAMES: Ataxin-7, Spinocerebellar ataxia type 7 protein, ATXN7, SCA7
- Protein Accession Number: O15265
- NCBI GENE ID NUMBER: 6314
- USER NOTE: Optimal dilutions for each application to be determined by the researcher.
Background
- The autosomal dominant cerebellar ataxias (ADCA) are aheterogeneous group of neurodegenerative disorders characterized byprogressive degeneration of the cerebellum, brain stem and spinalcord. Clinically, ADCA has been divided into three groups: ADCAtypes I-III. ADCAI is genetically heterogeneous, with five geneticloci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6,being assigned to five different chromosomes. ADCAII, which alwayspresents with retinal degeneration (SCA7), and ADCAIII oftenreferred to as the 'pure' cerebellar syndrome (SCA5), are mostlikely homogeneous disorders. Several SCA genes have been clonedand shown to contain CAG repeats in their coding regions. ADCA iscaused by the expansion of the CAG repeats, producing an elongatedpolyglutamine tract in the corresponding protein. The expandedrepeats are variable in size and unstable, usually increasing insize when transmitted to successive generations. This locus hasbeen mapped to chromosome 3, and it has been determined that thediseased allele associated with spinocerebellar ataxia-7 contains38-130 CAG repeats (near the N-terminus), compared to 7-17 in thenormal allele. The encoded protein is a component of theSPT3/TAF9/GCN5 acetyltransferase (STAGA) and TBP-freeTAF-containing (TFTC) chromatin remodeling complexes, and it thusplays a role in transcriptional regulation. Alternative splicingresults in multiple transcript variants.
- 1: Bonnet, J., et al. EMBO Rep. 11(8):612-618(2010)
- 2: Han, Y., et al. Neurol India 58(4):622-626(2010)
- 3: Chou, A.H., et al. Neurochem. Int. 56(2):329-339(2010)
- 4: Mookerjee, S., et al. J. Neurosci. 29(48):15134-15144(2009)
Disclaimer
- FOR RESEARCH USE ONLY
For additional information, visit ProSci's Terms & Conditions Page. - Disclaimer: Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
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