ATXN1 Antibody (PACO15843)
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주문- SKU:
- PACO15843
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
Antibody Name: | ATXN1 Antibody (PACO15843) |
Antibody SKU: | PACO15843 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:5000, WB:1:500-1:2000, IHC:1:50-1:200 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | Fusion protein of human ATXN1 |
Form: | Liquid |
Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Purification Method: | Antigen affinity purification |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
The image on the left is immunohistochemistry of paraffin-embedded Human liver cancer tissue using PACO15843(ATXN1 Antibody) at dilution 1/40, on the right is treated with fusion protein. (Original magnification: x200). | |
Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane 1-2: 293T cells, human fetal brain tissue, Primary antibody: PACO15843(ATXN1 Antibody) at dilution 1/800, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 20 seconds. | |
The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using PACO15843(ATXN1 Antibody) at dilution 1/40, on the right is treated with fusion protein. (Original magnification: x200). |
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. |
Synonyms: | ataxin 1 |
UniProt Protein Function: | ataxin-1: spinocerebellar ataxia type 1 (SCA1) protein. Expansion of CAG repeats in the SCA1 gene results in an abnormally long polyglutamine tract in the protein, causing polyglutamine-induced neurodegeneration. 14-3-3 and Akt kinase stabilize both wild-type and mutant forms of ataxin-1. The expansion of the polyglutamine tract may alter this function. |
UniProt Protein Details: | Protein type:Nuclear export; RNA-binding Chromosomal Location of Human Ortholog: 6p23 Cellular Component: cytoplasm; intracellular membrane-bound organelle; nuclear inclusion body; nuclear matrix; nuclear RNA export factor complex; nucleoplasm; nucleus Molecular Function:chromatin binding; DNA binding; identical protein binding; poly(rG) binding; poly(U) binding; protein binding; protein C-terminus binding; protein self-association Biological Process: adult locomotory behavior; alveolus development; negative regulation of insulin-like growth factor receptor signaling pathway; negative regulation of phosphorylation; negative regulation of transcription from RNA polymerase II promoter; negative regulation of transcription, DNA-dependent; nuclear export; positive regulation of transcription from RNA polymerase II promoter; regulation of excitatory postsynaptic membrane potential; RNA processing; transcription, DNA-dependent; visual learning Disease: Spinocerebellar Ataxia 1 |
NCBI Summary: | 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. [provided by RefSeq, Jan 2010] |
UniProt Code: | P54253 |
NCBI GenInfo Identifier: | 206729854 |
NCBI Gene ID: | 6310 |
NCBI Accession: | P54253.2 |
UniProt Secondary Accession: | P54253,Q17S02, Q9UJG2, Q9Y4J1, |
UniProt Related Accession: | P54253 |
Molecular Weight: | 86,923 Da |
NCBI Full Name: | Ataxin-1 |
NCBI Synonym Full Names: | ataxin 1 |
NCBI Official Symbol: | ATXN1 |
NCBI Official Synonym Symbols: | ATX1; SCA1; D6S504E |
NCBI Protein Information: | ataxin-1 |
UniProt Protein Name: | Ataxin-1 |
UniProt Synonym Protein Names: | Spinocerebellar ataxia type 1 protein |
Protein Family: | Ataxin |
UniProt Gene Name: | ATXN1 |
UniProt Entry Name: | ATX1_HUMAN |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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Anti-HRP-conjugated Beta Actin Antibody (CABC028) |