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DYNC1H1 Antibody, Biotin conjugated

  • 中文名稱：
    DYNC1H1兔多克隆抗體, Biotin偶聯(lián)
  • 貨號：
    CSB-PA613491LD01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) DYNC1H1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    DYNC1H1
  • 別名：
    DYNC1H1 antibody; DHC1 antibody; DNCH1 antibody; DNCL antibody; DNECL antibody; DYHC antibody; KIAA0325Cytoplasmic dynein 1 heavy chain 1 antibody; Cytoplasmic dynein heavy chain 1 antibody; Dynein heavy chain antibody; cytosolic antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Cytoplasmic dynein 1 heavy chain 1 protein (12-66AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Biotin
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.

產(chǎn)品評價

靶點詳情

  • 功能：
    Cytoplasmic dynein 1 acts as a motor for the intracellular retrograde motility of vesicles and organelles along microtubules. Dynein has ATPase activity; the force-producing power stroke is thought to occur on release of ADP. Plays a role in mitotic spindle assembly and metaphase plate congression.
  • 基因功能參考文獻：
    1. Taken together, these results demonstrate DYNC1H1 as a strong candidate and RTP1 as a potential candidate on the onset of epileptic encephalopathies. PMID: 28325891
    2. Here, the authors use quantitative imaging and laser ablation to show that NuMA targets dynactin to spindle microtubule minus-ends, localizing dynein activity there. PMID: 29185983
    3. Structural and functional mutations and hotspots for DYNC1H1. PMID: 28455235
    4. This observation offers an explanation for the dominant effects of DYNC1H1 mutations in vivo. PMID: 28196890
    5. This cohort demonstrates that mutations in DYNC1H1 can mimic a congenital myopathy. PMID: 28554554
    6. These results suggest that cytoplasmic dynein 1 binds to BRCA2 through the latter's centrosomal localization signal and BRCA2 mediates the cohesion between centrosomes during the S phase, potentially serving as a cell-cycle checkpoint. PMID: 27433848
    7. A novel de novo mutation (c.2327C > T, p.P776L) in the DYNC1H1 gene identified and confirmed it as the causal variant of Spinal muscular atrophy with lower extremity predominance. PMID: 26846447
    8. Our findings suggest that DYNC1H1 variants can cause not only lower, but also upper motor neuron disease. PMID: 26100331
    9. The mutations in DYNC1H1 increase the interaction with its adaptor BICD2. PMID: 25512093
    10. These findings also reveal a possible new target for Amblyomin-X, i.e., dynein, and may serve as a tool for investigating tumor cell death associated with proteasome inhibition. PMID: 25479096
    11. These results reveal that conformational changes involving hexon hypervariable region 1 are the basis for a novel viral mechanism controlling capsid transport to the nucleus by dynein. PMID: 25355895
    12. Report expands the clinical spectrum of DYNC1H1-related spinal muscular atrophy to include generalized arthrogryposis PMID: 25609763
    13. Authors propose that Snapin connects chlamydial inclusions with the microtubule network by interacting with both Chlamydia psittaci IncB and dynein. PMID: 24751478
    14. Authors find that pharmacological or small interfering RNA (siRNA)-mediated inhibition of cytoplasmic dynein or the kinesin 1 heavy chain KIF5B delays HIV-1 uncoating. PMID: 25231297
    15. single dynein molecules in the cell are autoinhibited through intramolecular head-head stacking PMID: 25266423
    16. Our results expand the set of pathological mutations in DYNC1H1, reinforce the role of cytoplasmic dynein in disorders of neuronal migration, and provide evidence for a syndrome including spinal nerve degeneration and brain developmental problems. PMID: 24307404
    17. This study demonistrated that DYNC1H1 mutation alters transport kinetics and ERK1/2-cFos signalling in a mouse model of distal spinal muscular atrophy. PMID: 24755273
    18. It focus on cytoplasmic dynein, which is required for a myriad of cellular functions in interphase, mitosis and meiosis, ranging from transport of organelles and functioning of the mitotic spindle to chromosome movements in meiotic prophase. PMID: 24256283
    19. In conclusion, association with microtubules and the translocation activity of dynein motor complexes are required to achieve efficient retrovirus restriction by TRIM5alpha. PMID: 24600008
    20. This study demonistrated that Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age. PMID: 23742762
    21. Data indicate that dynein- and astral microtubule-mediated transport of Galphai/LGN/nuclear mitotic apparatus (NuMA) complex from cell cortex to spindle poles. PMID: 23389635
    22. The cytoplasmic Dynein Heavy Chain 1 (DHC) was found to interact with NF1 along microtubules in vesicular structures identified to be melanosomes. PMID: 23583712
    23. Dynein forms distinct complexes requiring specific recruiters and activators to promote orderly progression through mitosis. PMID: 23589491
    24. this study has demonstrated that the same DYNC1H1 mutation could cause spinal muscular atrophy as well as distal neuropathy, indicating pleotropic effects of the mutation. PMID: 22847149
    25. analysis of reconstitution of the human cytoplasmic dynein complex PMID: 23213255
    26. study demonstrates that mutations in the tail domain of the heavy chain of cytoplasmic dynein (DYNC1H1) cause spinal muscular atrophy and provide experimental evidence that a DYNC1H1 mutation disrupts dynein complex assembly and function PMID: 22459677
    27. Mutations in DYNC1H1 can lead to a broad phenotypic spectrum, confirming the importance of DYNC1H1 in both central and peripheral neuronal functions. PMID: 22368300
    28. Exome sequencing of three affected individuals separated by eight meioses identified a single shared novel heterozygous variant, c.917A>G, in DYNC1H1, which encodes the cytoplasmic dynein heavy chain 1. PMID: 21820100
    29. In an in vitro MT gliding assay, both dynein-1 and dynein-2 showed minus-end-directed motor activities. PMID: 21723285
    30. mediates the perinuclear aggregation of phagocytosed melanosomes, participates in the formation of the supranuclear melanin cap or "microparasol" and serves as a mechanism to help protect the nucleus from ultraviolet-induced DNA damage. PMID: 14632200
    31. these results suggest that complexes of dynein, Lis1 and CLIP-170 crosslink and slide microtubules within the spindle, thereby producing an inward force that pulls centrosomes together. PMID: 19020519

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  • 相關(guān)疾病：
    Charcot-Marie-Tooth disease 2O (CMT2O); Mental retardation, autosomal dominant 13 (MRD13); Spinal muscular atrophy, lower extremity-predominant 1, autosomal dominant (SMALED1)
  • 亞細胞定位：
    Cytoplasm, cytoskeleton.
  • 蛋白家族：
    Dynein heavy chain family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 2961

    OMIM: 158600

    KEGG: hsa:1778

    STRING: 9606.ENSP00000348965

    UniGene: Hs.614080