Anti-ATXN7 Antibody (PACO55402)

Antibody Name:	ATXN7 Antibody (PACO55402)
Antibody SKU:	PACO55402
Size:	50ug
Host Species:	Rabbit
Tested Applications:	ELISA, WB, IHC, IF
Recommended Dilutions:	ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:300-1:500, IF:1:50-1:200
Species Reactivity:	Human, Rat
Immunogen:	Recombinant Human Ataxin-7 protein (402-503AA)

Form:	Liquid
Storage Buffer:	Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Purification Method:	>95%, Protein G purified
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Non-conjugated

	Western Blot. Positive WB detected in: Rat lung tissue. All lanes: ATXN7 antibody at 3.3µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 96, 102, 80 kDa. Observed band size: 80 kDa.
	Immunofluorescence staining of A549 cells with PACO55402 at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
	IHC image of PACO55402 diluted at 1:400 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

Background:	Acts as component of the STAGA transcription coactivator-HAT complex. Mediates the interaction of STAGA complex with the CRX and is involved in CRX-dependent gene activation. Necessary for microtubule cytoskeleton stabilization.
Synonyms:	Ataxin-7 (Spinocerebellar ataxia type 7 protein), ATXN7, SCA7

UniProt Protein Function:	ataxin-7: Acts as component of the STAGA transcription coactivator-HAT complex. Mediates the interaction of STAGA complex with the CRX and is involved in CRX-dependent gene activation. Necessary for microtubule cytoskeleton stabilization. Defects in ATXN7 are the cause of spinocerebellar ataxia type 7 (SCA7); also known as olivopontocerebellar atrophy III (OPCA III or OPCA3) or olivopontocerebellar atrophy with retinal degeneration. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA7 belongs to the autosomal dominant cerebellar ataxias type II (ADCA II) which are characterized by cerebellar ataxia with retinal degeneration and pigmentary macular dystrophy. Belongs to the ataxin-7 family. 2 isoforms of the human protein are produced by alternative splicing.Protein type: Nucleolus; Transcription regulationChromosomal Location of Human Ortholog: 3p21.1-p12Cellular Component: microtubule cytoskeleton; nucleoplasm; nuclear matrix; cytoplasm; nucleolus; nucleusMolecular Function: protein binding; chromatin bindingBiological Process: establishment and/or maintenance of chromatin architecture; transcription, DNA-dependent; visual perception; negative regulation of insulin-like growth factor receptor signaling pathway; negative regulation of phosphorylation; positive regulation of transcription from RNA polymerase II promoter; microtubule cytoskeleton organization and biogenesis; nuclear organization and biogenesis; histone deubiquitinationDisease: Spinocerebellar Ataxia 7
UniProt Protein Details:
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. This locus has been mapped to chromosome 3, and it has been determined that the diseased allele associated with spinocerebellar ataxia-7 contains 38-130 CAG repeats (near the N-terminus), compared to 7-17 in the normal allele. The encoded protein is a component of the SPT3/TAF9/GCN5 acetyltransferase (STAGA) and TBP-free TAF-containing (TFTC) chromatin remodeling complexes, and it thus plays a role in transcriptional regulation. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2010]
UniProt Code:	O15265
NCBI GenInfo Identifier:	5921169
NCBI Gene ID:	6314
NCBI Accession:	O15265.1
UniProt Secondary Accession:	O15265,O75328, O75329, Q9Y6P8, B4E207, E9PHP9
UniProt Related Accession:	O15265
Molecular Weight:	79,535 Da
NCBI Full Name:	Ataxin-7
NCBI Synonym Full Names:	ataxin 7
NCBI Official Symbol:	ATXN7
NCBI Official Synonym Symbols:	SCA7; OPCA3; ADCAII
NCBI Protein Information:	ataxin-7; spinocerebellar ataxia type 7 protein
UniProt Protein Name:	Ataxin-7
UniProt Synonym Protein Names:	Spinocerebellar ataxia type 7 protein
Protein Family:	Ataxin
UniProt Gene Name:	ATXN7
UniProt Entry Name:	ATX7_HUMAN

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)