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Recombinant Human ATP-binding cassette sub-family A member 3 (ABCA3), partial

  • 中文名稱:
    Recombinant Human ATP-binding cassette sub-family A member 3(ABCA3) ,partial
  • 貨號:
    CSB-YP859942HU
  • 規格:
  • 來源:
    Yeast
  • 其他:
  • 中文名稱:
    Recombinant Human ATP-binding cassette sub-family A member 3(ABCA3) ,partial
  • 貨號:
    CSB-EP859942HU
  • 規格:
  • 來源:
    E.coli
  • 其他:
  • 中文名稱:
    Recombinant Human ATP-binding cassette sub-family A member 3(ABCA3) ,partial
  • 貨號:
    CSB-EP859942HU-B
  • 規格:
  • 來源:
    E.coli
  • 共軛:
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他:
  • 中文名稱:
    Recombinant Human ATP-binding cassette sub-family A member 3(ABCA3) ,partial
  • 貨號:
    CSB-BP859942HU
  • 規格:
  • 來源:
    Baculovirus
  • 其他:
  • 中文名稱:
    Recombinant Human ATP-binding cassette sub-family A member 3(ABCA3) ,partial
  • 貨號:
    CSB-MP859942HU
  • 規格:
  • 來源:
    Mammalian cell
  • 其他:

產品詳情

  • 純度:
    >85% (SDS-PAGE)
  • 基因名:
    ABCA3
  • Uniprot No.:
  • 別名:
    ABC 3; ABC C; ABC C transporter; ABC transporter 3; ABC-C transporter; ABC3; ABCA 3; Abca3; ABCA3 protein; ABCA3_HUMAN; ABCC; ATP binding cassette 3; ATP binding cassette sub family A (ABC1) member 3; ATP binding cassette sub family A member 3; ATP binding cassette transporter 3; ATP-binding cassette 3; ATP-binding cassette sub-family A member 3; ATP-binding cassette transporter 3; CED7. C. elegans; homolog of; EST111653; LBM 180; LBM180; MGC72201; P180 Lamellar Body Protein; SMDP3
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Partial
  • 蛋白標簽:
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式:
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶:
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期:
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期:
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項:
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet :
    Please contact us to get it.

產品評價

靶點詳情

  • 功能:
    Catalyzes the ATP-dependent transport of phospholipids such as phosphatidylcholine and phosphoglycerol from the cytoplasm into the lumen side of lamellar bodies, in turn participates in the lamellar bodies biogenesis and homeostasis of pulmonary surfactant. Transports preferentially phosphatidylcholine containing short acyl chains. In addition plays a role as an efflux transporter of miltefosine across macrophage membranes and free cholesterol (FC) through intralumenal vesicles by removing FC from the cell as a component of surfactant and protects cells from free cholesterol toxicity.
  • 基因功能參考文獻:
    1. a mutation (E292V) located in the first cytoplasmic loop of ABCA3 did not significantly affect lipid transport, but rather resulted in smaller vesicles. In addition to these findings, the assay used in this work for analysing the PC-lipid transport into ABCA3 positive vesicles will be useful to screen for compounds susceptible to restore function in mutated ABCA3 protein. PMID: 28887056
    2. two ABCA3 mutations (p.R288K and p.R1474W) identified among term and late-preterm infants with respiratory distress syndrome, were characterized. PMID: 27374344
    3. 1153 patients with diffuse parenchymal lung disease (DPLD) were registered in the KLR. The DNA of 242 of these patients was sequenced for ABCA3 mutations. 69 patients had at least one variation in the ABCA3 gene. Of 40 patients with two disease-causing ABCA3 mutations, 22 patients were homozygous and 18 heterozygous. PMID: 27516224
    4. Genes ABCC7, A3, A8, A12, and C8 prevailed among the most upregulated or downregulated ones. In conclusion, the results supported our theory about general adenosine triphosphate-binding cassette gene expression profiles and their importance for cancer on clinical as well as research levels. PMID: 28468577
    5. transporter oligomerization is crucial for ABCA3 function. PMID: 27352740
    6. The TGGAG haplotype may be a risk factor for Respiratory Distress Syndrome in preterm infants in this Chinese population. The haplotype TGGAG was significantly more frequent in RDS infants than in non-RDS infants. PMID: 26522252
    7. Rare mutations in surfactant-associated genes contribute to neonatal respiratory distress syndrome. We resequenced all exons of the ATP-binding cassette member A3 (ABCA3) and we found three ABCA3 mutations in the Han [minor allele frequency (MAF)=0.003] and 7 in the Zhuang (MAF=0.011) cohorts. The contribution of these rare ABCA3 mutations to disease burden in the south China population is still unknown. PMID: 26547207
    8. The clinical features of ABCA3 mutations, including onset, severity or clinical course are very heterogeneous. In the two siblings we had a lightly discordant course that could be explained by exposure to different environmental stresses or variable penetrance. PMID: 26508177
    9. These results provide evidence of ABCA3 as an MLF efflux transporter in human macrophages and support its role in the direct antileishmanial effect of this alkylphosphocholine drug. PMID: 26903515
    10. Studies indicate that ATP-binding cassette (ABC), subfamily A, member 3 (ABCA3) is developmentally regulated. PMID: 26517903
    11. Data discussed the structural features of ABCA3 and how the use of bioinformatics tools could help researchers to obtain a reliable structural model to locate relevant mutations and make genotype/phenotype correlations of affected patients. [review] PMID: 26295388
    12. SLCO1B3 699GG and 344TT genotypes are associated with non-response to IM, while ABCA3 4548-91 CC/CA genotypes are related to poor CMR in CML patients treated with standard-dose imatinib. PMID: 25056761
    13. Accumulation of free cholesterol as a result of a loss of ABCA3 export function represents a novel pathomechanism in ABCA3-induced Diffuse parenchymal lung disease. PMID: 25817392
    14. Report of ABCA3 mutations in a family with one child exhibiting interstitial lung disease. PMID: 23846195
    15. In the title. PMID: 25073622
    16. We identified a cataract-microcornea syndrome (cmcc) associated gene, ABCA3, which had heterozygous missense mutations in two autosomal dominant CCMC families. Another four heterozygous mutations, 2 missense and 2 splice site mutations were identified. PMID: 25406294
    17. Therapeutic strategies for chronic interstitial lung disease have been used successfully in cases of a mild clinical course in juvenile patients with ABCA3 gene mutation. In our patient with homozygous ABCA3 gene mutation,they were not effective. PMID: 24633979
    18. Two siblings are described who were homozygous for a 5,983 bp deletion in ABCA3 including exons 2-5 as well as the start AUG codon and a putative Golgi exit signal motif. PMID: 24420869
    19. A large kindred is identified with a novel ABCA3 mutation causing pulmonary fibrosis. PMID: 24730976
    20. ABCA3 protein, human genetic variants does not increase the risk of neonatal respiratory distress syndrome. PMID: 24657120
    21. Genotype-phenotype correlations exist for homozygous or compound heterozygous mutations in ABCA3 causing neonatal respiratory failure or childhood interstitial lung disease. PMID: 24871971
    22. A novel missense mutation in ABCA3 was found to cause fatal congenital surfactant deficiency in two siblings. PMID: 24628317
    23. Identification of novel compound heterozygous mutations in the coding exons of ABCA3 in two brothers with interstitial lung disease. PMID: 23443156
    24. A compound heterozygote for both novel mutations in the ABCA3 gene. PMID: 24269975
    25. cotranslational N-linked glycosylation at N124 and N140 is critical for ABCA3 stability, and its disruption results in protein destabilization and proteasomal degradation PMID: 24142515
    26. Tyrosine kinase inhibitor exposure facilitates a protective loop of SALL4 and ABCA3 cooperation in persistent leukaemic cells. PMID: 23432194
    27. partially reduced ABCA3 activity due to E292V is not a major risk factor for reduced lung function and COPD in the general population PMID: 22866751
    28. Genetic variants within ABCA3 may be the genetic cause of or a contributor to some unexplained refractory neonatal respiratory distress syndrome. PMID: 22455634
    29. Although ABCA3 mutations are individually rare, they are collectively common among European- and African-descent individuals in the general population. PMID: 23166334
    30. An intronic ABCA3 mutation is responsible for a fatal respiratory disease in newborns. PMID: 22337229
    31. There is an association between a synonymous cSNP rs323043 and the development of neonatal respiratory distress syndrome. PMID: 22800827
    32. identification of new ABCA3 mutations in patients with life-threatening neonatal respiratory distress and/or pediatric interstitial lung disease (ILD); two mutations associated with ILD acted via different pathophysiological mechanisms despite similar clinical phenotypes PMID: 22068586
    33. Data suggest the impairment of epithelial function as a mechanism by which ABCA3 mutations cause interstitial lung disease (ILD). PMID: 22434821
    34. the ABCA3 missense mutation E292V had no remarkable effect on pulmonary outcome in VLBW infants. Present results do not rule out the possibility that E292V phenotype is associated with minor difference in the morbidity. PMID: 22145626
    35. Several genetic abnormalities have been associated with familial pulmonary fibrosis. The present study examined the genes coding for surfactant protein-C, ATPbinding cassette protein A3 and telomerase, and found no abnormalities. PMID: 21165348
    36. A novel conserved targeting motif found in ABCA transporters mediates trafficking to early post-Golgi compartments. PMID: 21586796
    37. Lymphoma exosomes shield target cells from antibody attack and that exosome biogenesis is modulated by the lysosome-related organelle-associated ATP-binding cassette (ABC) transporter A3 (ABCA3). PMID: 21873242
    38. SALL4 was able to bind to the promoter region of ABCA3 and activate its expression while regulating the expression of ABCG2 indirectly. PMID: 21526180
    39. Suggest that expression of partially or completely endoplasmic reticulum localized ABCA3 mutant proteins can increase the apoptotic cell death of the affected cells. PMID: 21214890
    40. Data show that the ABCA3 N-terminus is proteolytically removed inside acidic LAMP3-positive vesicles MVB/LB. PMID: 20863830
    41. The segregation of ABCA3 alleles, absence of ABCA3 immunostaining, lung pathology, and ultrastructural findings support genetic ABCA3 deficiency as the cause of lung disease. PMID: 20304423
    42. The identification of one copy of this novel mutation in a premature infant with chronic respiratory insufficiency suggests that ABCA3 haploinsufficiency together with lung prematurity may result in more severe, or more prolonged, respiratory failure PMID: 19861431
    43. Subclinical fibrotic changes may be present in family members of patients with SFTPC mutation-associated interstitial lung disease and suggest ABCA3 variants could affect disease pathogenesis. PMID: 20371530
    44. Decreased ABCA3 expression is associated with breast cancer. PMID: 19902402
    45. Identification of LBM180, a lamellar body limiting membrane protein of alveolar type II cells, as the ABC transporter protein ABCA3 PMID: 11940594
    46. Results suggest that ABCA3 shows ATPase activity, which is induced by lipids, and may be involved in the biogenesis of lamellar body-like structures. PMID: 15465012
    47. ABCA3 is required for lysosomal loading of phosphatidylcholine and conversion of lysosomes to lamellar body-like structures PMID: 16415354
    48. ABCA3 has a role in drug resistance in childhood acute myeloid leukemia PMID: 16857811
    49. ABCA3 mutation is associated with fatal surfactant deficiency PMID: 16959783
    50. ABCA17P & ABCA3 form a complex of overlapping genes at their 5' ends. Non-coding & protein-coding ABC A-transporter RNAs are expressed. This is the 1st demonstration of expression of a pseudogene & its parent from a common overlapping human DNA region. PMID: 16968533

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  • 相關疾病:
    Pulmonary surfactant metabolism dysfunction 3 (SMDP3)
  • 亞細胞定位:
    Endosome, multivesicular body membrane; Multi-pass membrane protein. Cytoplasmic vesicle membrane. Late endosome membrane. Lysosome membrane.
  • 蛋白家族:
    ABC transporter superfamily, ABCA family
  • 組織特異性:
    Expressed in brain, pancreas, skeletal muscle and heart. Highly expressed in the lung in an AT2-cell-specific manner. Weakly expressed in placenta, kidney and liver. Also expressed in medullary thyroid carcinoma cells (MTC) and in C-cell carcinoma.
  • 數據庫鏈接:

    HGNC: 33

    OMIM: 601615

    KEGG: hsa:21

    STRING: 9606.ENSP00000301732

    UniGene: Hs.26630



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