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Wichtige Dokumente
A3361
Anti-ATP13A2 (C-terminal region) antibody produced in rabbit
~1.5 mg/mL, affinity isolated antibody, buffered aqueous solution
Synonym(e):
Anti-ATPase type 13A2, Anti-PARK9
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About This Item
UNSPSC-Code:
12352203
NACRES:
NA.41
Empfohlene Produkte
Biologische Quelle
rabbit
Qualitätsniveau
Konjugat
unconjugated
Antikörperform
affinity isolated antibody
Antikörper-Produkttyp
primary antibodies
Klon
polyclonal
Form
buffered aqueous solution
Mol-Gew.
antigen ~129 kDa
Speziesreaktivität
mouse, human
Erweiterte Validierung
recombinant expression
Learn more about Antibody Enhanced Validation
Konzentration
~1.5 mg/mL
Methode(n)
western blot: 1.5-3.0 μg/mL using mouse brain extract (S1 fraction) or HEK-293T cells expressing human ATP13A2
UniProt-Hinterlegungsnummer
Versandbedingung
dry ice
Lagertemp.
−20°C
Posttranslationale Modifikation Target
unmodified
Angaben zum Gen
human ... ATP13A2(23400)
Allgemeine Beschreibung
ATP13A2 (ATPase type 13A2, also known as PARK9) is a neuronal P-type ATPase of the P5 subfamily. It is present in the lysosome of transiently transfected cells, whereas the unstable truncated mutants are retained in the endoplasmic reticulum and degraded by the proteasome.
ATP13A2 is a member of the P5 subfamily of P-type transport ATPases which include ATP13A1-ATP13A5. Mutations in ATP3A2 also known as PARK9 are associated with hereditary Parkinson′s disease.
Rabbit anti-ATP13A2 (C-terminal region) antibody is specific for human and mouse ATP13A2. Staining of the ATP13A2 band by immunoblotting is specifically inhibited by the ATP13A2 immunizing peptide.
Anwendung
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Western Blotting (1 paper)
Western Blotting (1 paper)
Rabbit anti-ATP13A2 (C-terminal region) antibody has been used for western blotting applications at a dilution of 1:1000.
Biochem./physiol. Wirkung
ATP13A2 (ATPase type 13A2, also known as PARK9) shows elevated expression levels in the brains of sporadic Parkinson′s disease (PD) patients, suggesting a potential role in the more common forms of PD. It is associated with Kufor-Rakeb syndrome (KRS). KRS is a rare form of hereditary PD with juvenile onset. In addition to typical signs of PD, affected individuals show symptoms of more widespread pyramidal neurodegeneration, including dementia.
Physikalische Form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
Haftungsausschluss
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Lagerklassenschlüssel
12 - Non Combustible Liquids
WGK
nwg
Flammpunkt (°F)
Not applicable
Flammpunkt (°C)
Not applicable
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David Ramonet et al.
Human molecular genetics, 21(8), 1725-1743 (2011-12-22)
Mutations in the ATP13A2 gene (PARK9, OMIM 610513) cause autosomal recessive, juvenile-onset Kufor-Rakeb syndrome and early-onset parkinsonism. ATP13A2 is an uncharacterized protein belonging to the P(5)-type ATPase subfamily that is predicted to regulate the membrane transport of cations. The physiological
Mark Ainsley Colijn et al.
Neurogenetics (2024-07-18)
Biallelic (autosomal recessive) pathogenic variants in ATP13A2 cause a form of juvenile-onset parkinsonism, termed Kufor-Rakeb syndrome. In addition to motor symptoms, a variety of other neurological and psychiatric symptoms may occur in affected individuals, including supranuclear gaze palsy and cognitive
Aaron M Gusdon et al.
Neurobiology of disease, 45(3), 962-972 (2011-12-27)
Mitochondrial dysfunction and autophagy are centrally implicated in Parkinson's disease (PD). Mutations in ATP13A2, which encodes a lysosomal P-type ATPase of unknown function, cause a rare, autosomal recessive parkinsonian syndrome. Lysosomes are essential for autophagy, and autophagic clearance of dysfunctional
Hereditary parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase
Ramirez A, et al.
Nature Genetics, 38(10), 1184-1184 (2006)
Alejandro Estrada-Cuzcano et al.
Brain : a journal of neurology, 140(2), 287-305 (2017-02-01)
Hereditary spastic paraplegias are heterogeneous neurodegenerative disorders characterized by progressive spasticity of the lower limbs due to degeneration of the corticospinal motor neurons. In a Bulgarian family with three siblings affected by complicated hereditary spastic paraplegia, we performed whole exome
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