Rabbit anti-ATXN1 Antibody

ATXN1 pAb

$320.00  
144-66089-100
+

Antigen Information

Accession Number:
  • P54253
Gene ID:
  • 6310
Gene Symbols:
  • ATXN1
Protein Name & Synonyms:
ATXN1, ATX1, D6S504E, SCA1, ataxin-1
Target Species:
  • Human

Antibody Specifications

Applications:
WB, IF
Buffer:
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Conjugation/Tag:
Unconjugated
Host Species:
Rabbit
Purity:
Affinity purification
Species Reactivity:
Human, Mouse, Rat
Isotype:
IgG

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.

Dilutions

WB 1:500 - 1:2000; IF 1:10 - 1:100

Format

Calculated MW
86kDa
Observed MW
87kDa

Immunogen

Recombinant fusion protein containing a sequence corresponding to amino acids 586-815 of human ATXN1 (NP_001121636.1).

Modification

Unmodified

Storage

Store at -20°. Avoid freeze / thaw cycles.

Images

Western blot

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Usage

This product is furnished for LABORATORY RESEARCH USE ONLY.
Not for diagnostic or therapeutic use.

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