YRP04812-01 [Polyclonal Antibody]
ATXN3L Rabbit Polyclonal Antibody
www.yhsbio.com
market@yhsbio.com
support@yhsbio.com
+86-21-54651191
Room 703,Building 6,333# Guiping
Rd.,Xuhui District,Shanghai,China
DATASHEET
Species:   Rabbit
Applications:   WB IHC IF
Immunogen Range:   KLH conjugated synthetic peptide derived from human ATXN3L
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:   92552
Swiss Prot:  
Synonyms:   ATX3L_HUMAN, ATXN3L, Machado-Joseph disease protein 1-like, MJDL, Putative ataxin-3-like protein.
Purification:   Purified by Protein A.
Storage:   Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months
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.
Caculated MW:   /
Observed MW:   Refer to Figures
Applications:   WB 1:100-1:1000
IHC 1:100-1:500
IF 1:50-1:200
Reacitivity:   Human
For research use only. Not intended for diagnostic or therapeutic use!
Additional information