anti-Human Ataxin 1 Polyclonal Antibody | anti-ATXN1 antibody

Anti-Ataxin 1 Antibody

Cat. Num. AAA178222

• Gene Names: ATXN1; ATX1; SCA1; D6S504E
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: Immunogen Affinity Purified
• Synonyms: Ataxin 1; Polyclonal Antibody; Anti-Ataxin 1 Antibody; Ataxin-1; ATX1; ATX1_HUMAN; Atxn1; D6S504E; OTTHUMP00000016065; SCA1; Spinocerebellar ataxia type 1 protein antibody; ataxin 1; anti-ATXN1 antibody

• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Immunogen Affinity Purified
• Form/Format: Lyophilized. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Sequence Length: 815

• Applicable Applications for anti-ATXN1 antibody: Western Blot (WB), Immunohistochemistry (IHC) Paraffin
• Application Notes: Western Blot Concentration: 0.1-0.5ug/ml; Immunohistochemistry (IHC) Paraffin Concentration: 0.5-1ug/ml
• Immunogen: A synthetic peptide corresponding to a sequence at the C-terminus of human Ataxin 1 (778-808aa ESRKLEKSEDEPPLTLPKPSLIPQEVKICIE), different from the related mouse and rat sequences by one amino acid.
• Ig Type: Rabbit IgG
• Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
• Preparation and Storage: At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquoted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

(Anti- Ataxin 1 Picoband antibody, MBS178222, Western blottingAll lanes: Anti Ataxin 1 (MBS178222) at 0.5ug/mlLane 1: MCF-7 Whole Cell Lysate at 40ugLane 2: COLO320 Whole Cell Lysate at 40ugLane 3: PANC Whole Cell Lysate at 40ugPredicted bind size: 87KDObserved bind size: 87KD )

Immunohistochemistry (IHC)

(Anti- Ataxin 1 Picoband antibody, MBS178222, IHC(P)IHC(P): Human Lung Cancer Tissue )

Related Product Information for anti-ATXN1 antibody

Description: Rabbit IgG polyclonal antibody for Ataxin-1(ATXN1) detection. Tested with WB, IHC-P in Human.

Background: Ataxin-1 is a protein that in humans is encoded by the ATXN1 gene. The ATXN1 gene had been mapped to 6p23 by in situ hybridization. Ataxin-1 (ATXN1), a causative factor for spinocerebellar ataxia type 1 (SCA1), and the related Brother of ATXN1 (BOAT1) are human proteins involved in transcriptional repression. ATXN1 and BOAT1 might participate in several Notch-controlled developmental and pathological processes.

References

1. "Entrez Gene: ATXN1 ataxin 1" 2. Tong, X., Gui, H., Jin, F., Heck, B. W., Lin, P., Ma, J., Fondell, J. D., Tsai, C.-C. Ataxin-1 and brother of ataxin-1 are components of the Notch signalling pathway. EMBO Rep. 12: 428-435, 2011. 3. Volz A, Fonatsch C, Ziegler A (Jun 1992). "Regional mapping of the gene for autosomal dominant spinocerebellar ataxia (SCA1) by localizing the closely linked D6S89 locus to 6p24.2----p23.05". Cytogenet Cell Genet 60 (1): 37-9. 4. Volz, A., Boyle, J. M., Cann, H. M., Cottingham, R. W., Orr, H. T., Ziegler, A. Report of the second international workshop on human chromosome 6. Genomics 21: 464, 1994.

NCBI and Uniprot Product Information

• NCBI GI #: 51479158
• NCBI GeneID: 6310
• NCBI Accession #: NP_000323.2
• NCBI GenBank Nucleotide #: NM_000332.3
• UniProt Accession #: P54253; Q17S02; Q9UJG2; Q9Y4J1
• Molecular Weight: 86,923 Da
• NCBI Official Full Name: ataxin-1
• NCBI Official 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
• Protein Family: Ataxin
• UniProt Gene Name: ATXN1
• UniProt Synonym Gene Names: ATX1; SCA1
• UniProt Entry Name: ATX1_HUMAN

NCBI Description

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 Description

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.

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: DNA binding; identical protein binding; poly(rG) binding; poly(U) binding; protein binding; protein C-terminus binding; protein self-association

Biological Process: negative regulation of transcription, DNA-dependent; nuclear export; RNA processing; transcription, DNA-dependent

Disease: Spinocerebellar Ataxia 1