Description:

Size: 100ul

Catalog no.: bs-4807R-A594

Price: 380 EUR

Product details

Modification Site

None

Gene ID Number

92552

Crossreactivity

Human

Target Antigen

ATXN3L

Tested applications

IF(IHC-P)

French translation

anticorps

Clonality

Polyclonal

Modification

Unmodified

Concentration

1ug per 1ul

Excitation emission

590nm/617nm

Conjugated with

ALEXA FLUOR® 594

Conjugated

Alexa conjugate 1

Clone

Polyclonal antibody

Recommended dilutions

IF(IHC-P)(1:50-200)

Purification

Purified by Protein A.

Category

Conjugated Primary Antibodies

Conjugation

Alexa Fluor,ALEXA FLUOR® 594

Host Organism

Rabbit (Oryctolagus cuniculus)

Also known as

Anti-ATXN3L PAb ALEXA FLUOR 594

Specificity

This is a highly specific antibody against ATXN3L.

Long name

ATXN3L Polyclonal Antibody, ALEXA FLUOR 594 Conjugated

Cross-reactive species details

Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

Synonyms

ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.

Source

This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human ATXN3L

Storage conditions

Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.

Properties

For facs or microscopy Alexa 1 conjugate.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

Background of the antigen

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.