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ATP13A2 Antibody

  • 货号：
    CSB-PA23379A0Rb
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunofluorescence staining of A549 cells with CSB-PA23379A0Rb at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ATP13A2 Polyclonal antibody
  • Uniprot No.：
    Q9NQ11
  • 基因名：
    ATP13A2
  • 别名：
    AT132_HUMAN antibody; Atp13a2 antibody; ATPase type 13A2 antibody; CLN12 antibody; FLJ26510 antibody; HSA9947 antibody; KRPPD antibody; PARK9 antibody; Probable cation transporting ATPase 13A2 antibody; Probable cation-transporting ATPase 13A2 antibody; Putative ATPase antibody; RP1-37C10.4 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Cation-transporting ATPase 13A2 protein (582-736AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ATP13A2 Antibody (CSB-PA23379A0Rb)，的标记方式是Non-conjugated。对于ATP13A2 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA23379B0Rb ATP13A2 Antibody, HRP conjugated ELISA
    FITC CSB-PA23379C0Rb ATP13A2 Antibody, FITC conjugated
    Biotin CSB-PA23379D0Rb ATP13A2 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    ATPase which acts as a lysosomal polyamine exporter with high affinity for spermine. Also stimulates cellular uptake of polyamines and protects against polyamine toxicity. Plays a role in intracellular cation homeostasis and the maintenance of neuronal integrity. Contributes to cellular zinc homeostasis. Confers cellular protection against Mn(2+) and Zn(2+) toxicity and mitochondrial stress. Required for proper lysosomal and mitochondrial maintenance. Regulates the autophagy-lysosome pathway through the control of SYT11 expression at both transcriptional and post-translational levels. Facilitates recruitment of deacetylase HDAC6 to lysosomes to deacetylate CTTN, leading to actin polymerization, promotion of autophagosome-lysosome fusion and completion of autophagy. Promotes secretion of exosomes as well as secretion of SCNA via exosomes. Plays a role in lipid homeostasis.
  • 基因功能参考文献：
    1. This study showed that Lysosomal defects in ATP13A2 associated familial Parkinson's disease. PMID: 28894968
    2. Hereditary Parkinsonism-associated genetic variations in PARK9 locus lead to functional impairment in the ion transport function of this protein. (Review) PMID: 26965689
    3. Study unravels a novel activity-independent scaffolding role of ATP13A2 in trafficking/export of intracellular cargo in response to proteotoxic stress. PMID: 28334751
    4. we describe, for the first time, the deleterious effect arising from the interaction between the ATP13A2 familial mutant Dup22 with a-Syn. We show that this ATP13A2 mutant can enhance a-Syn oligomerization and aggregation in cell culture PMID: 27282395
    5. ATP13A2 inhibition by hsa-miR-4306 efficiently restored manganese-induced cytotoxicity in cultured neurons. PMID: 28302480
    6. This study demonstrated that loss of ATP13A2 function causes a combination of lysosomal and mitochondrial dysfunction that affects multiple neuronal populations. PMID: 28137957
    7. The ATP13A2 A746T variant is rare in Han Chinese patients and controls and is not associated with PD susceptibility in this ethnic group. PMID: 26000924
    8. This study showed that LRRK2, PARK2 and ATP13A2 are under copy number variations influence in patient with Parkinson disease. PMID: 27399248
    9. tre results of this study suggests that the expression of ATP13A2 regulated by the PHD2-HIF1alpha signaling pathway,and this is instrumental in maintaining cellular iron homeostasis and cell viability in mitochondrially compromised DAergic neurons. PMID: 26818499
    10. that ATP13A2 contains a unique N-terminal hydrophobic extension that lies on the cytosolic membrane surface of the lysosome, where it interacts with the lysosomal signaling lipids phosphatidic acid and phosphatidylinositol(3,5)bisphosphate. PMID: 26134396
    11. ATP13A2 overexpression improves the lysosome membrane integrity and protects against iron-induced cell damage. PMID: 25912790
    12. A review of recent advances in the emerging association of ATP13A2 mutations with Parkinsonism and neuronal ceroid lipofuscinoses. PMID: 25197640
    13. The mutation rates of Thr12Met and Ala1144Thr of ATP13A2 in the Uygur and Han Parkinson's disease patients in the Xinjiang region are low. PMID: 25374329
    14. This study demonistrated that loss of ATP13A2 causes a specific protein trafficking defect, and that Atp13a2 null mice develop age-related motor dysfunction that is preceded by neuropathological changes. PMID: 25855184
    15. Data show that patients with Lewy body diseases have an overall deficit in ATP13A2 protein levels, with the remaining protein being more insoluble and partially redistributing towards Lewy bodies PMID: 24252509
    16. Present results of homozygosity mapping in two siblings affected with early onset Parkinson's disease (EOPD) and mutation screening of ATP13A2. The variation identified represents the 13th known disease causing mutation in ATP13A2. PMID: 24949580
    17. these data suggest the involvement of PARK9 in the biogenesis of exosomes and alpha-synuclein secretion. PMID: 25392495
    18. Reduced ATP13A2 expression significantly decreased vesicular zinc levels, indicating ATP13A2 facilitates transport of zinc. PMID: 24603074
    19. human ATP13A2 deficiency results in zinc dyshomeostasis and mitochondrial dysfunction. PMID: 24399444
    20. PARK9 loss of function leads to dyshomeostasis of intracellular Zn(2+) that in turn contributes to lysosomal dysfunction and accumulation of alpha-Syn. PMID: 24334770
    21. No association is found between ATP13A2 Ala746Thr and early onset Parkinson's disease (EOPD) or late onset Parkinson's in a Chinese cohort; this variant is not a strong risk factor in the Chinese population. PMID: 23522931
    22. hypoxia signaling plays a very important role in the regulation of human ATP13A2 gene expression PMID: 22288903
    23. a novel frame-shift mutation in exon 22 of ATP13A2 (c.2473C>AA, p.Leu825AsnfsX32). PMID: 21696388
    24. Mutations in ATP13A2 is often associated with rapidly progressive parkinsonism and with additional features including pyramidal signs, cognitive decline and loss of sustained Levodopa responsiveness. PMID: 23196729
    25. Cathepsin D activity was decreased in ATP13A2-knockdown cells that displayed lysosome-like bodies characterized by fingerprint-like structures PMID: 23499937
    26. ATP13A2 knockout exacerbates apoptosis and autophagy in infarct penumbra of cerebral cortex, with no influence on the infarct size in mice with ischemic stroke. PMID: 23121889
    27. ATP13A2 protects against manganese & nickel toxicity, & proteasomal, mitochondrial, & oxidative stress. ATP13A2 may import a cofactor required for the function of a lysosome enzyme(s). PMID: 22847264
    28. the human P5B-ATPase ATP13A2 is involved in polyamine uptake. PMID: 23205587
    29. ATP13A2 variation may be a risk marker for neurotoxic effects of manganese in humans. PMID: 22285144
    30. ATP13A2 and alpha-syn are functionally linked in neurodegeneration. PMID: 22645275
    31. Our results indicate that ATP13A2 mutations are a rare cause of both NBIA and dystonia-parkinsonism. PMID: 22743658
    32. This study provides support for common loss-of-function effects of homozygous and heterozygous missense mutations in ATP13A2 associated with early-onset forms of parkinsonism. PMID: 22768177
    33. The altered apoptotic pattern of subjects with mutated ATP13A2 suggests a correlation between apoptosis alteration and the mutated ATP13A2 protein PMID: 22117566
    34. These findings collectively suggest that ATP13A2 contributes to the maintenance of a healthy mitochondrial pool, supporting the hypothesis that impaired mitochondrial clearance represents an important pathogenic mechanism underlying KRS. PMID: 22296644
    35. Data show that a family with typical neuronal ceroid lipofuscinoses (NCLs) pathology carried a single homozygous mutation in ATP13A2 that fully segregates with disease. PMID: 22388936
    36. This study demonistrated that contralateral silent period duration was increased in the symptomatic ATP13A2 mutation carriers suggested that compound heterozygous mutation in the ATP13A2 gene is associated with increased intracortical inhibition. PMID: 22104014
    37. results unravel an instrumental role of ATP13A2 deficiency on lysosomal function and cell viability and demonstrate the feasibility and therapeutic potential of modulating ATP13A2 levels in the context of PD PMID: 22647602
    38. results confirm a role for Ypk9 in manganese homeostasis and illuminates cellular pathways and biological processes in which Ypk9 likely functions PMID: 22457822
    39. The SNPs investigated in the BST1, PARK15 and PARK9 genes associated with PD susceptibility are not associated with PD in the northern Han Chinese population. PMID: 22490479
    40. study reveals a number of intriguing neuronal phenotypes due to the loss- or gain-of-function of ATP13A2 that support a role for this protein in regulating intracellular cation homeostasis and neuronal integrity PMID: 22186024
    41. This study demonistrated that restoration of ATP13A2 function may lead to improved lysosomal function and decreased accumulation of alpha-syn. PMID: 22442086
    42. rare variants of ATP13A2 may contribute to Parkinson's disease susceptibility in Taiwan PMID: 21714071
    43. Mutant Atp13a2 proteins are degraded by endoplasmic reticulum-associated degradation and sensitize cells to cell death. PMID: 21665991
    44. A novel frameshift mutation in ATP13A2 causes juvenile dystonia-parkinsonism and dementia. PMID: 21094623
    45. premature degradation of mutant ATP13A2 mRNA contribute to the aetiology of Kufor-Rakeb syndrome (KRS). PMID: 21542062
    46. Regulation of intracellular manganese homeostasis by Kufor-Rakeb syndrome-associated ATP13A2 protein. PMID: 21724849
    47. To see if ATP13A2 mutations could be responsible for some cases of human adult-onset NCL (Kufs disease), we resequenced ATP13A2 from 28 Kufs disease patients. None of these patients had ATP13A2 sequence variants likely to be causal for their disease PMID: 21362476
    48. No clearly pathogenic mutations are identified in ATP13A2 and GIGYF2 in Brazilian patients with early-onset Parkinson's disease. PMID: 20816920
    49. report clinical, instrumental, and genetic findings in an Italian family with novel PARK9 and PARK15 mutations PMID: 20853184
    50. We found no evidence for a correlation between a single heterozygous mutation in the ATP13a2 gene and the development of distinct oculomotor disturbances. PMID: 20842691

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  • 相关疾病：
    Kufor-Rakeb syndrome (KRS); Spastic paraplegia 78, autosomal recessive (SPG78)
  • 亚细胞定位：
    Lysosome membrane; Multi-pass membrane protein. Late endosome membrane; Multi-pass membrane protein. Endosome, multivesicular body membrane; Multi-pass membrane protein. Cytoplasmic vesicle, autophagosome membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Cation transport ATPase (P-type) (TC 3.A.3) family, Type V subfamily
  • 组织特异性：
    Expressed in brain; protein levels are markedly increased in brain from subjects with Parkinson disease and subjects with dementia with Lewy bodies. Detected in pyramidal neurons located throughout the cingulate cortex (at protein level). In the substanti
  • 数据库链接：

    HGNC: 30213

    OMIM: 606693

    KEGG: hsa:23400

    STRING: 9606.ENSP00000327214

    UniGene: Hs.128866