DYNC1H1 Antibody, FITC conjugated

說明書

中文名稱：

DYNC1H1兔多克隆抗體, FITC偶聯
貨號：

CSB-PA613491LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) DYNC1H1 Polyclonal antibody
Uniprot No.：

Q14204
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：

FITC
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
產品提供形式：

Liquid
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

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
數據庫鏈接：

HGNC: 2961

OMIM: 158600

KEGG: hsa:1778

STRING: 9606.ENSP00000348965

UniGene: Hs.614080

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