Product Name :
Ataxin 1 Rabbit pAb
Cat.No.:
db3317
Synonyms :
ATX1; SCA1; D6S504E
Application :
WB, IP
Reactivity :
Human, Mouse, Rat
Host species :
Rabbit
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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, Mouse, Rat
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Application
WB, IP
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Recommended dilution
WB: 1:1000
IP: 1:20
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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
Polyclonal
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Isotype
IgG
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Purity
Affinity Purification
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Conjugation
Un-conjugated
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Storage/Stability
Store at -20°C. Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.
订购信息
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Package
Price (RMB)
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20μL
598
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50μL
998
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100μL
1798
- 货期：现货
For research use only. Not intended for diagnostic.
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