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Mouse Recombinant Anti-ATXN7 Recombinant Antibody (1C1) (CBMAB-XB0071-YC)

Provided herein is a Mouse Recombinant Antibody against Ataxin 7. The antibody can be used for immunoassay techniques, such as IF, WB.
See all ATXN7 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
1C1
Antibody Isotype
IgG1, κ
Application
WB, ICC, ELISA

Basic Information

Immunogen
Recombinant protein (1-229).
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IF(ICC)1:500-1:5,000
ELISA1:500-1:5,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Ascites
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Ataxin 7
Introduction
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 37-306 CAG repeats (near the N-terminus), compared to 4-35 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.
Entrez Gene ID
Human6314
Mouse246103
UniProt ID
HumanO15265
MouseQ8R4I1
Alternative Names
Ataxin 7; Spinocerebellar Ataxia Type 7 Protein; SCA7; Spinocerebellar Ataxia 7 (Olivopontocerebellar Atrophy With Retinal Degeneration); Ataxin-7; ADCAII; OPCA3;
Function
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.
Biological Process
Histone deubiquitination Source: UniProtKB
Microtubule cytoskeleton organization Source: UniProtKB
Negative regulation of microtubule depolymerization Source: CACAO
Nucleus organization Source: ProtInc
Protein deubiquitination Source: Reactome
Visual perception Source: ProtInc
Cellular Location
Isoform a: Cytoskeleton; Nucleus; Nucleolus; Nucleus matrix. In addition to a diffuse distribution throughout the nucleus, it is associated with the nuclear matrix and the nucleolus. It is able to shuttle between the nucleus and cytoplasm.
Isoform b: Cytoplasm
Involvement in disease
Spinocerebellar ataxia 7 (SCA7): 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.
PTM
Proteolytically cleaved. The cleavage may be involved in SCA7 degeneration: the isoform fragments may exert distinct toxic influences that could contribute to selective neurodegeneration.
Sumoylation decreases the aggregation propensity and cellular toxicity of forms with an expanded poly-Gln region but has no effect on subcellular location or interaction with components of the STAGA complex.

Niss, F., Zaidi, W., Hallberg, E., & Ström, A. L. (2021). Polyglutamine expanded Ataxin-7 induces DNA damage and alters FUS localization and function. Molecular and Cellular Neuroscience, 110, 103584.

Kotowska-Zimmer, A., Ostrovska, Y., & Olejniczak, M. (2020). Universal RNAi triggers for the specific inhibition of mutant huntingtin, atrophin-1, ataxin-3, and ataxin-7 expression. Molecular Therapy-Nucleic Acids, 19, 562-571.

Dela Peña, I. J. I., Botanas, C. J., de la Peña, J. B., Custodio, R. J., Dela Peña, I., Ryoo, Z. Y., ... & Cheong, J. H. (2019). The Atxn7-overexpressing mice showed hyperactivity and impulsivity which were ameliorated by atomoxetine treatment: A possible animal model of the hyperactive-impulsive phenotype of ADHD. Progress in Neuro-Psychopharmacology and Biological Psychiatry, 88, 311-319.

Niss, F., Zaidi, W., Papadopoulou, E., Hallberg, E., & Ström, A. L. (2019). Polyglutamine expanded Ataxin-7 alters FUS localization and function in a SCA7 cell model.

Carrillo-Rosas, S., Weber, C., Fievet, L., Messaddeq, N., Karam, A., & Trottier, Y. (2019). Loss of zebrafish Ataxin-7, a SAGA subunit responsible for SCA7 retinopathy, causes ocular coloboma and malformation of photoreceptors. Human molecular genetics, 28(6), 912-927.

Marinello, M., Werner, A., Giannone, M., Tahiri, K., Alves, S., Tesson, C., ... & Sittler, A. (2019). SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models. Disease models & mechanisms, 12(1), dmm036145.

Cloud, V., Thapa, A., Morales-Sosa, P., Miller, T. M., Miller, S. A., Holsapple, D., ... & Mohan, R. D. (2019). Ataxin-7 and Non-stop coordinate SCAR protein levels, subcellular localization, and actin cytoskeleton organization. Elife, 8, e49677.

Carrillo-Rosas, S. (2017). Role of Ataxin-7 in the development of vertebrate eye and its impact in the understanding of human eye pathologies and spinocerebellar ataxia type 7 (Doctoral dissertation, Université de Strasbourg).

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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