PARK9 (Probable Cation-transporting ATPase 13A2, ATP13A2)

Parkinson's disease (PD), the second most common neurodegenerative disease after Alzheimer's, is a progressive movement disorder characterized by rigidity, tremors and postural instability. The pathological hallmark of PD is progressive loss of dopaminergic neurons in the substantia nigra of the ventral midbrain and the pres- ence of intracellular Lewy bodies (protein aggregates of a-synuclein, ubiquitin and other components) in surviving neurons of the brain stem (1). Various genes and loci (a-synuclein/PARK1 and 4, parkin/PARK2, UCH-L1/PARK5, PINK1/PARK6, DJ-1/PARK7, LRRK2/PARK8, ATP13A2/ PARK9) are genetically linked to PD (2). PARK9, also known as ATP13A2, is a member of the P-type ATPase superfamily and is involved in the lysosomal degradation pathway, clearing a-synuclein aggregates (3,4). The protein has 10 transmembrane domains and wild-type PARK9 localizes to the lysosomal membrane. In contrast, all three known mutations, which have premature stop codons resulting in a truncated protein, are retained in the endo- plasmic reticulum and degraded by the proteasome. PARK9 is predominantly expressed in the brain and has been linked to Kufor-Rakeb Syndrome, a monogenic form of recessively inherited, atypical parkinsonism that is characterized by juvenile onset, with pyramidal degeneration and cognitive dysfunction (5,6).\n\nApplications: \nSuitable for use in Western Blot and Immunoprecipitation. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:1000\nImmunoprecipitation: 1:50\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

P3111-10

Size

100ul

Applications

IP, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat, NHP

Form

Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 100ug/ml BSA, 50% glycerol.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

1.Fahn, S. (2003) Ann N Y Acad Sci 991, 1-14. 2.Moore, D.J. et al. (2005) Annu Rev Neurosci 28, 57-87. 3.Ramirez, A. et al. (2006) Nat Genet 38, 1184-91. 4.Xiromerisiou, G. et al. (2010) Neurosurg Focus 28, E7. 5.Klein, C. and Lohmann-Hedrich, K. (2007) Curr Opin Neurol 20, 453-64. 6.Xiromerisiou, G. et al. (2010) Neurosurg Focus 28, E7.

Additional Info

Recognizes endogenous levels of total human PARK9. Species Crossreactivity: Monkey, mouse and rat.