84-1885-95　［取扱停止］ATXN1 (S776) (Ataxin-1, Spinocerebellar Ataxia Type 1 Protein, ATX1, SCA1) (Azide free) (HRP) 200ul　A4159-04A-HRP

特徴

• 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.
• Applications:Suitable for use in ELISA and Western Blot.
• Other applications not tested.
• Recommended Dilution:ELISA: 1:1,000Western Blot: 1:50-1:100Optimal 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.