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, and is associated with spinocerebellar ataxia type 1 (SCA1).
At least two transcript variants encoding the same protein have been found for this gene.
Applications:Suitable for use in ELISA.
Other applications have not been tested.
Recommended Dilutions:Optimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：100ul
Host：goat
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by peptide affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. Labeled with Horseradish peroxidase (HRP).
Specificity：Recognizes ATXN1.
Isotype：IgG
Calc Applications Abbrev：E
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to C-DLEVQQATHREASP from the internal region of human ATXN1. Species sequence homology: human, canine, porcine
