ATXN3L antibody (AA 251-355) (AbBy Fluor® 555)

Catalog No. ABIN1396642

Product Details anti-ATXN3L Antibody (AbBy Fluor® 555)

Target: ATXN3L (Ataxin 3-Like (ATXN3L))

Binding Specificity: AA 251-355

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This ATXN3L antibody is conjugated to AbBy Fluor® 555

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Cross-Reactivity: Human

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human ATXN3L

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for ATXN3L

Target: ATXN3L (Ataxin 3-Like (ATXN3L))

Alternative Name: ATXN3L (ATXN3L Products)

Synonyms: MJDL antibody, ataxin 3 like antibody, ATXN3L antibody

Background:

Synonyms: ATX3L_HUMAN, ATXN3L, Machado-Joseph disease protein 1-like, MJDL, Putative ataxin-3-like protein.

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.

Gene ID: 92552