ATXN1 (clone 2F5), anti-human
€394.00
In stock
SKU
253104
Catalog Nr.: 253104
Size: 0.1 ml
Isotype: Mouse IgG1
Applications: WB
Size: 0.1 ml
Isotype: Mouse IgG1
Applications: WB
Protein Family: Transcription Factors
Pathway and Disease: Transcription, Neurodegenerative Disorders
Description:
Ataxin-1 is a chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Ataxin-1 is related to autosomal dominant cerebellar ataxias (ADCA). ADCA is 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. 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. Ataxin-1 is widely expressed throughout the body.
Alternate Names: Ataxin-1, Spinocerebellar ataxia type 1 protein, ATX1, SCA1, ATXN1
Application Notes: WB: 1:500-1:2000
Accession No.: P54253
Antigen: Purified recombinant fragment of human ATXN1 expressed in E. coli.
Format: Each vial contains 0.1 ml ascitic fluid with 0.03% sodium azide.
Storage:
Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months from the date of shipment.
Pathway and Disease: Transcription, Neurodegenerative Disorders
Description:
Ataxin-1 is a chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Ataxin-1 is related to autosomal dominant cerebellar ataxias (ADCA). ADCA is 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. 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. Ataxin-1 is widely expressed throughout the body.
Alternate Names: Ataxin-1, Spinocerebellar ataxia type 1 protein, ATX1, SCA1, ATXN1
Application Notes: WB: 1:500-1:2000
Accession No.: P54253
Antigen: Purified recombinant fragment of human ATXN1 expressed in E. coli.
Format: Each vial contains 0.1 ml ascitic fluid with 0.03% sodium azide.
Storage:
Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months from the date of shipment.
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