Ataxin 1 (DGR14428) Rabbit mAb (PBS Only)
Product Name :
Ataxin 1 (DGR14428) Rabbit mAb (PBS Only)
Cat.No.:
db12665-PBS
Synonyms :
ATX1; SCA1; D6S504E
Reactivity :
Human
Host species :
Rabbit
Conjugation/Formulation:PBS Only
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Background
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 40-83 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. [provided by RefSeq, Jul 2016]
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Immunogen
Recombinant protein of human Ataxin 1
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Gene ID
6310
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Swiss Prot
P54253
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Synonyms
ATX1; SCA1; D6S504E
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Reactivity
Human
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Application
WB, IP
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Recommended dilution
WB: 1:1000-1:5000
IP: 1:20-1:50
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Calculated MW
87 kDa
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Observed MW
105 kDa
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Host species
Rabbit
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Clonality
Monoclonal
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Clonality No.
DGR14428
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Isotype
IgG
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Purity
Affinity Purification
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Conjugation
Un-conjugated
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Concentration
1 mg/ml
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Formulation
PBS Only
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Storage/Stability
Store at -20°C. Recommended to aliquot into single-use vials. Supplied in 1X PBS (pH 7.4). BSA and Azide Free. Stable for 12 months from date of receipt.
×
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Western blot detection of Ataxin 1 in 3T3 cell lysates using
db12665(1:1000 diluted).
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Western blot analysis of extracts from T24,C2C12 cells using
db12665
订购信息
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Package
Price (RMB)
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100μg
5798
- 货期:现货
For research use only. Not intended for diagnostic.