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Antikörper

ATXN1 Antibody

Produktgrößen
100ul
£359,00
OASG00594-100UL
Über dieses Produkt
SKU:
OASG00594
Zusätzliche Namen:
ATX1; SCA1; D6S504E
Klonalität:
Polyclonal
Konzentration:
1 mg/ml
Weitere Details:
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 41-81 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.
Gendetails:
ataxin 1
Host:
Rabbit
Immunogen:
Synthesized peptide derived from human Ataxin-1 around the phosphorylation site of S776. Location: 720-800aa.
Isotyp:
IgG
Molekulargewicht:
87 kDa
Aufreinigung:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Versandbedingungen:
Blue Ice
Lagerbedingungen:
Store at -20C. Avoid repeated freeze/thaw cycles.
Hersteller:
Aviva Systems Biology
Typ:
Antibodies: Polyclonal Antibody