DNM1L Antibody
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中文名稱：DNM1L兔多克隆抗體
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貨號：CSB-PA007078GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：DLP1 antibody; dnm1l antibody; DNM1L_HUMAN antibody; Dnm1p/Vps1p-like protein antibody; dnml1 antibody; DRP1 antibody; DVLP antibody; Dymple antibody; Dynamin 1 like antibody; Dynamin family member proline-rich carboxyl-terminal domain less antibody; Dynamin like protein antibody; Dynamin related protein 1 antibody; Dynamin-1-like protein antibody; Dynamin-like protein 4 antibody; Dynamin-like protein antibody; Dynamin-like protein IV antibody; Dynamin-related protein 1 antibody; DYNIV 11 antibody; EMPF antibody; EMPF1 antibody; FLJ41912 antibody; HdynIV antibody; VPS1 antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human DNM1L,DLP1
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Functions in mitochondrial and peroxisomal division. Mediates membrane fission through oligomerization into membrane-associated tubular structures that wrap around the scission site to constrict and sever the mitochondrial membrane through a GTP hydrolysis-dependent mechanism. The specific recruitment at scission sites is mediated by membrane receptors like MFF, MIEF1 and MIEF2 for mitochondrial membranes. While the recruitment by the membrane receptors is GTP-dependent, the following hydrolysis of GTP induces the dissociation from the receptors and allows DNM1L filaments to curl into closed rings that are probably sufficient to sever a double membrane. Acts downstream of PINK1 to promote mitochondrial fission in a PRKN-dependent manner. Plays an important role in mitochondrial fission during mitosis. Through its function in mitochondrial division, ensures the survival of at least some types of postmitotic neurons, including Purkinje cells, by suppressing oxidative damage. Required for normal brain development, including that of cerebellum. Facilitates developmentally regulated apoptosis during neural tube formation. Required for a normal rate of cytochrome c release and caspase activation during apoptosis; this requirement may depend upon the cell type and the physiological apoptotic cues. Required for formation of endocytic vesicles. Proposed to regulate synaptic vesicle membrane dynamics through association with BCL2L1 isoform Bcl-X(L) which stimulates its GTPase activity in synaptic vesicles; the function may require its recruitment by MFF to clathrin-containing vesicles. Required for programmed necrosis execution. Rhythmic control of its activity following phosphorylation at Ser-637 is essential for the circadian control of mitochondrial ATP production.; Inhibits peroxisomal division when overexpressed.; Inhibits peroxisomal division when overexpressed.
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基因功能參考文獻：
- cryo-electron microscopy structure of full-length human DRP1 co-assembled with MID49 and an analysis of structure- and disease-based mutations PMID: 29899447
- Data show that increasing dynamin-related protein 1 (Drp1) SUMOylation by knocking down SUMO1-sentrin-SMT3 specific protease 3 (SENP3) reduces both Drp1 binding to mitochondrial fission factor protein (Mff) and stress-induced cytochrome c release. PMID: 28262828
- knockdown of LRP6 inhibited the cell viability by activation of Drp1 in glucose deprived-cardiomyocytes. PMID: 29864925
- Results suggest that the loss of dynamin-related protein 1 (Drp1) expression could contribute to the development of lung and colon cancers. PMID: 29329364
- observations indicate that homozygous p.T115M variant of DNM1L produces a neurological and neurodevelopmental phenotype, consistent with impaired mitochondrial architecture and function, through a diminished ability to oligomerize, which was most prevalent under oxidative stress. PMID: 29110115
- Study results reveal a crucial function for Drp1 in regulating tumor growth, mitochondrial morphology, and cell cycle in cutaneous squamous cell carcinoma. PMID: 28818497
- Elimination of Drp1 by shRNA or Mdivi-1 (a Drp1-specific inhibitor) suppressed GBP2's regulatory function. Furthermore, GBP2 blocks Drp1 translocation from the cytosol to mitochondria, thereby attenuating Drp1-dependent mitochondrial fission and breast cancer cell invasion. PMID: 29072687
- hyperacetylation of microtubules contributes to the recruitment of total Drp1 to mitochondria to enhance fission. PMID: 28757354
- Results uncovered a novel mechanism of Drp1-mediated mitochondrial fragmentation in senecionine-induced liver injury. PMID: 28282614
- miR-21-5p/203a-3p promote ox-LDL-induced endothelial senescence through down-regulation of Drp1 in a direct or indirect way. PMID: 28347692
- this study shows that Drp1 can impact survival of epithelial ovarian cancer patients PMID: 27509055
- The structure and function of DNM1L protein in mitochondrial fission is reviewed. PMID: 28132464
- Results described a recessive disease caused by DNM1L mutations, with a clinical phenotype resembling mitochondrial disorders but without any typical biochemical features. Two novel DNM1L mutations (one frame-shift mutation and one missense mutation) are identified and was found to be associated with impaired mitochondrial and peroxisomal morphology. PMID: 27328748
- Study describes mutations in ZNF143 causing a previously undescribed inherited disorder of vitamin B12 (cobalamin) metabolism. These mutations cause an accumulation of transcobalamin-bound cobalamin within the cells, as well as decreased expression of MMACHC, a cobalamin trafficking protein. PMID: 27349184
- The results suggest that endoplasmic reticulum (ER) can function as a platform for Drp1 oligomerization, and that ER-associated Drp1 contributes to mitochondrial division. PMID: 29158231
- PRKAA deletion promoted mitochondrial fragmentation in vascular endothelial cells by inhibiting the autophagy-dependent degradation of DNM1L. PMID: 28085543
- hepatic stimulator substance could regulate mitochondrial fission and hepatocyte apoptosis during liver ischemia/reperfusion injury by orchestrating the translocation and activation of Drp1 PMID: 28646508
- This report describes a patient with a DNM1L mutation and abnormalities in mitochondrial fission and function. The pathogenicity and the dominant nature of the novel p.G362S mutation are demonstrated by overexpression of the mutant gene. PMID: 26992161
- In contrast to the initial report of neonatal lethality resulting from DNM1L mutation and DRP1 dysfunction, our results show that milder DRP1 impairment is compatible with normal early development and subsequently results in a distinct set of neurological findings. In addition, we identify a common pathogenic mechanism whereby DNM1L mutations impair mitochondrial fission. PMID: 27145208
- These findings provide new insights into MCL-1 ligands, and the interplay between DRP-1 and the anti-apoptotic BCL-2 family members in the regulation of apoptosis PMID: 28079887
- High drp1 expression is associated with cisplatin-induced apoptosis of renal tubular epithelial cells. PMID: 28423497
- This study demonstrated that Mutations in DNM1L, as in OPA1, result indominant optic atrophy despite opposite effectson mitochondrial fusion and fission. PMID: 28969390
- Modulation of mitochondrial fission by increased levels of pDrp1 S616. PMID: 28388446
- This is the first study to identify an association between SIRT4 expression and decreased mitochondrial fission, which was driven by Drp1. SIRT4 inhibited Drp1 phosphorylation and weakened Drp1 recruitment to the mitochondrial membrane via an interaction with Fis-1. PMID: 27941873
- the mitochondrial morphology of T-cell acute lymphoblastic leukemia cells were altered from elongation to fragmentation because of the extracellular signal-regulated kinase activation-mediated phosphorylation of the pro-fission factor, dynamin-related protein 1 (Drp1), at residue S616. PMID: 27831567
- DNM1L was found to be involved in the regulation of collagen secretion and cardiovascular calcification. PMID: 28607103
- The authors determine that Dengue virus nonstructural protein (NS)4B, a promising drug target with unknown function, associates with mitochondrial proteins, including Drp1, and alters mitochondria morphology to promote infection. PMID: 27545046
- Depletion of septin 2 reduces Drp1 recruitment to mitochondria and results in hyperfused mitochondria and delayed FCCP-induced fission. PMID: 27215606
- Missense variants in the middle domain of DNM1L is associated with infantile encephalopathy. PMID: 26931468
- Genetic silencing of Drp1 increases mitochondrial proton leak in MIN6 cells. Drp1 does not control insulin secretion via its effect on proton leak but instead via modulation of glucose-fueled respiration. PMID: 28174288
- DNM1L missense mutation identified in a patient with developmental delay, refractory epilepsy and prolonged survival. Patient fibroblasts showed striking hyperfusion of the mitochondrial network. Bioenergetic studies in patient fibroblasts showed no significant differences versus controls. PMID: 26604000
- Disruption of Drp1 and subsequent mitochondrial fragmentation events prevents impaired vascular dilation, restores mitochondrial phenotype, and implicates mitochondrial fission as a primary mediator of endothelial dysfunction PMID: 27923790
- MiD49 and MiD51 recruit inactive forms of Drp1 in mitochondrial fission. [review] PMID: 27660309
- FUNDC1 integrates mitochondrial fission and mitophagy at the interface of the endoplasmic reticulum-mitochondrial contact site by working in concert with DRP1 and calnexin under hypoxic conditions in mammalian cells. PMID: 27145933
- This study reveals an essential role of SUMOylated FADD in Drp1- and caspase-10-dependent necrosis. PMID: 27799292
- Sustained phosphorylation of Akt by Abeta directly activates Drp1 and inhibits autophagy through the mTOR pathway. Together, these changes elicit abundant mitochondrial fragmentation resulting in ROS-mediated neuronal apoptosis. PMID: 27599716
- Drp1 was decreased on mitochondria during Dengue virus infection, as well as Drp1 phosphorylated on serine 616, which is important for mitochondrial fission. PMID: 27816895
- These data suggest a model for ARSACS where neurons with reduced levels of sacsin are compromised in their ability to recruit or retain Drp1 at the mitochondrial membrane leading to a decline in mitochondrial health, potentially through impaired mitochondrial quality control. PMID: 27288452
- Taken together, our data demonstrate that DRG2 acts as a regulator of mitochondrial fission by controlling the expression of Drp1. PMID: 28363867
- results lend further support to the notion that VPS35-DLP1 interaction is key to the retromer-dependent recycling of mitochondrial DLP1 complex during mitochondrial fission and provide a novel therapeutic target to control excessive fission and associated mitochondrial deficits. PMID: 28040727
- Mitochondrial morphology and cellular distribution are altered in SPG31 patients and are linked to DRP1 hyperphosphorylation. PMID: 28007911
- the mitochondrial division factor Dnm1 in yeast or Drp1 in mammalian cells is dispensable for mitophagy. PMID: 27903607
- Silencing Drp1 inhibits glioma cells proliferation and invasion by RHOA/ ROCK1 pathway. PMID: 27495873
- that miR-30a could inhibit TET1 expression through base pairing with complementary sites in the 3'untranslated region to regulate Drp-1 promoter hydroxymethylation. PMID: 28294974
- Improper transcriptional (in)activation of mitofusin-1 and dynamin-related protein 1 during early in vitro embryo development is associated with a decrease in mitochondrial membrane potential and with embryo fragmentation. PMID: 25033890
- this study reveals coordinated increase of mitochondrial biogenesis and mitophagy in which Drp1 plays a central role regulating breast cancer cell metabolism and survival. PMID: 27746856
- Dynamin-related protein 1 (Dpr1) activates mitochondrial-dependent apoptosis and indicate that inhibiting Dpr1 function can protect against chlorpyrifos-induced cytotoxicity. PMID: 26598294
- Drp1 is in dynamic equilibrium on mitochondria in a fission-independent manner, and that fission factors such as actin filaments target productive oligomerization to fission sites. PMID: 26609810
- Dindings demonstrate for the first time, that Drp1 is required for Bax mitochondrial translocation, but Drp1-induced mitochondrial fragmentation alone is not sufficient to induce apoptosis in DLBCL cells. PMID: 26093086
- Haploinsufficiency of Drp1 abolished mitochondrial autophagy and exacerbated the development of heart failure. PMID: 26915633
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相關疾病：Encephalopathy due to defective mitochondrial and peroxisomal fission 1 (EMPF1)
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亞細胞定位：Cytoplasm, cytosol. Golgi apparatus. Endomembrane system; Peripheral membrane protein. Mitochondrion outer membrane; Peripheral membrane protein. Peroxisome. Membrane, clathrin-coated pit. Cytoplasmic vesicle, secretory vesicle, synaptic vesicle membrane.
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蛋白家族：TRAFAC class dynamin-like GTPase superfamily, Dynamin/Fzo/YdjA family
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組織特異性：Ubiquitously expressed with highest levels found in skeletal muscles, heart, kidney and brain. Isoform 1 is brain-specific. Isoform 2 and isoform 3 are predominantly expressed in testis and skeletal muscles respectively. Isoform 4 is weakly expressed in b
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