ATXN3L Polyclonal Antibody 
Datasheet
COA
MSDS
SHIP
Product Name :
ATXN3L Polyclonal Antibody Background :
Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). 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. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease. Product :
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
ATXN3L Polyclonal Antibody detects endogenous levels of ATXN3L protein. Immunogen :
KLH conjugated synthetic peptide derived from human ATXN3L Conjugate :
Unconjugated Modification :
Unmodification
ATXN3L Polyclonal Antibody Background :
Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). 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. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease. Product :
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
ATXN3L Polyclonal Antibody detects endogenous levels of ATXN3L protein. Immunogen :
KLH conjugated synthetic peptide derived from human ATXN3L Conjugate :
Unconjugated Modification :
Unmodification
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Primary: Anti-ATXN3L at 1/1000 dilution
Bioworld Biotech only provide peptides for our antibodies and do not provide additional peptide customization services.
Price/Size :
USD 368/1mg/vial
Tips:
For phospho antibody, we provide phospho peptide（0.5mg) and non-phospho peptide(0.5mg).Describe :
Blocking peptides are peptides that bind specifically to the target antibody and block antibody binding. These peptide usually contains the epitope recognized by the antibody. Antibodies bound to the blocking peptide no longer bind to the epitope on the target protein. This mechanism is useful when non-specific binding is an issue, for example, in Western blotting (WB) and Immunohistochemistry (IHC). By comparing the staining from the blocked antibody versus the antibody alone, one can see which staining is specific; Specific binding will be absent from the western blot or IHC performed with the neutralized antibody.Formula:
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 10 mg/ml.The purity is >90%,tested by HPLC and MS.
Storage:
The freeze-dried powder is more stable. For short time at 2-8°C. For long term storage store at -20°C.
Note :
This product is for research use only (RUO only). Not for use in diagnostic or therapeutic procedures.