Haploinsufficiency of the TDP43 ubiquitin E3 ligase RNF220 leads to ALS-like motor neuron defects in the mouse.

Abstract:

:TDP43 pathology is seen in a large majority of amyotrophic lateral sclerosis (ALS) cases, suggesting a central pathogenic role of this regulatory protein. Clarifying the molecular mechanism controlling TDP43 stability and subcellular location might provide important insights into ALS therapy. The ubiquitin E3 ligase RNF220 is involved in different neural developmental processes through various molecular targets in the mouse. Here, we report that the RNF220+/- mice showed progressively decreasing mobility to different extents, some of which developed typical ALS pathological characteristics in spinal motor neurons, including TDP43 cytoplasmic accumulation, atrocytosis, muscle denervation, and atrophy. Mechanistically, RNF220 interacts with TDP43 in vitro and in vivo and promotes its polyubiquitination and proteasomal degradation. In conclusion, we propose that RNF220 might be a modifier of TDP43 function in vivo and contribute to TDP43 pathology in neurodegenerative disease like ALS.

journal_name

J Mol Cell Biol

authors

Ma P,Li Y,Wang H,Mao B

doi

10.1093/jmcb/mjaa072

subject

Has Abstract

pub_date

2021-01-02 00:00:00

eissn

1674-2788

issn

1759-4685

pii

6059226

pub_type

杂志文章
  • Mdm2 as a chromatin modifier.

    abstract::Mdm2 is the key negative regulator of the tumour suppressor p53, making it an attractive target for anti-cancer drug design. We recently identified a new role of Mdm2 in gene repression through its direct interaction with several proteins of the polycomb group (PcG) family. PcG proteins form polycomb repressive comple...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章,评审

    doi:10.1093/jmcb/mjw046

    authors: Wienken M,Moll UM,Dobbelstein M

    更新日期:2017-02-01 00:00:00

  • The long and the short of it: the MDM4 tail so far.

    abstract::The mouse double minute 4 (MDM4) is emerging from the shadow of its more famous relative MDM2 and is starting to steal the limelight, largely due to its therapeutic possibilities. MDM4 is a vital regulator of the tumor suppressor p53. It restricts p53 transcriptional activity and also, at least in development, facilit...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章,评审

    doi:10.1093/jmcb/mjz007

    authors: Haupt S,Mejía-Hernández JO,Vijayakumaran R,Keam SP,Haupt Y

    更新日期:2019-03-01 00:00:00

  • Fenton reactions drive nucleotide and ATP syntheses in cancer.

    abstract::We present a computational study of tissue transcriptomic data of 14 cancer types to address: what may drive cancer cell division? Our analyses point to that persistent disruption of the intracellular pH by Fenton reactions may be at the root of cancer development. Specifically, we have statistically demonstrated that...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjy039

    authors: Sun H,Zhang C,Cao S,Sheng T,Dong N,Xu Y

    更新日期:2018-10-01 00:00:00

  • SP1 governs primordial folliculogenesis by regulating pregranulosa cell development in mice.

    abstract::Establishment of the primordial follicle (PF) pool is pivotal for the female reproductive lifespan; however, the mechanism of primordial folliculogenesis is poorly understood. Here, the transcription factor SP1 was shown to be essential for PF formation in mice. Our results showed that SP1 is present in both oocytes a...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjz059

    authors: Cai H,Liu B,Wang H,Sun G,Feng L,Chen Z,Zhou J,Zhang J,Zhang T,He M,Yang T,Guo Q,Teng Z,Xin Q,Zhou B,Zhang H,Xia G,Wang C

    更新日期:2020-04-24 00:00:00

  • Molecular mechanisms of ligand-mediated attenuation of DNA binding by MarR family transcriptional regulators.

    abstract::Bacteria and archaea encode members of the large multiple antibiotic resistance regulator (MarR) family of transcriptional regulators. Generally, MarR homologs regulate activity of genes involved in antibiotic resistance, stress responses, virulence or catabolism of aromatic compounds. They constitute a diverse group ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjq021

    authors: Perera IC,Grove A

    更新日期:2010-10-01 00:00:00

  • A role for paralog-specific sumoylation in histone deacetylase 1 stability.

    abstract::Histone deacetylase 1 (HDAC1) is an essential epigenetic regulator belonging to a highly conserved family of deacetylases. Increased HDAC1 activity and expression often correlates with neoplastic transformation. Here we show how specific modification of HDAC1 by SUMO1, but not by SUMO2, facilitates HDAC1 degradation. ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjt032

    authors: Citro S,Jaffray E,Hay RT,Seiser C,Chiocca S

    更新日期:2013-12-01 00:00:00

  • TIAR and TIA-1 mRNA-binding proteins co-aggregate under conditions of rapid oxygen decline and extreme hypoxia and suppress the HIF-1α pathway.

    abstract::T-cell intracellular antigen (TIA)-1 and TIA-1-related protein (TIAR) are mRNA-binding proteins that can aggregate within granules under specific stress conditions. In this study, we analyzed TIAR/TIA-1 aggregation under different hypoxic conditions, and studied the effects on the hypoxia-inducible factor (HIF)-1α in ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjq032

    authors: Gottschald OR,Malec V,Krasteva G,Hasan D,Kamlah F,Herold S,Rose F,Seeger W,Hänze J

    更新日期:2010-12-01 00:00:00

  • Embryonic hematopoiesis in vertebrate somites gives rise to definitive hematopoietic stem cells.

    abstract::Hematopoietic stem cells (HSCs) replenish all types of blood cells. It is debating whether HSCs in adults solely originate from the aorta-gonad-mesonephros (AGM) region, more specifically, the dorsal aorta, during embryogenesis. Here, we report that somite hematopoiesis, a previously unwitnessed hematopoiesis, can gen...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjw024

    authors: Qiu J,Fan X,Wang Y,Jin H,Song Y,Han Y,Huang S,Meng Y,Tang F,Meng A

    更新日期:2016-08-01 00:00:00

  • Ablation of Zfhx4 results in early postnatal lethality by disrupting the respiratory center in mice.

    abstract::Breathing is an integrated motor behavior that is driven and controlled by a network of brainstem neurons. Zfhx4 is a zinc finger transcription factor and our results showed that it was specifically expressed in several regions of the mouse brainstem. Mice lacking Zfhx4 died shortly after birth from an apparent inabil...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjaa081

    authors: Zhang M,Du S,Ou H,Cui R,Jiang N,Lin Y,Ge R,Ma D,Zhang J

    更新日期:2021-01-20 00:00:00

  • Novel proteasome inhibitors as potential drugs to combat tuberculosis.

    abstract::Mycobacterium tuberculosis is one of the most notorious killers worldwide. These pathogens have evolved to infect human beings in a so-called dormant form that is extremely difficult to treat. New work, however, suggests that mycobacterial proteasomes, multicomponent structures that protect the microbe from damaging e...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjp053

    authors: Cheng Y,Pieters J

    更新日期:2010-08-01 00:00:00

  • BMP signaling promotes the growth of primary human colon carcinomas in vivo.

    abstract::Human colon carcinomas (CCs) represent a growing worldwide problem. One of the pathways that has been negatively implicated in the genesis of CCs is triggered by bone morphogenetic protein (BMP) ligands, which activate BMP receptors leading to the function of SMAD proteins in the nucleus. BMP signaling is altered in f...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjq035

    authors: Lorente-Trigos A,Varnat F,Melotti A,Ruiz i Altaba A

    更新日期:2010-12-01 00:00:00

  • Cancer and neurodegenerative disorders: pathogenic convergence through microRNA regulation.

    abstract::Although cancer and neurodegenerative disease are two distinct pathological disorders, emerging evidence indicates that these two types of disease share common mechanisms of genetic and molecular abnormalities. Recent studies show that individual microRNAs (miRNAs) could be involved in the pathology of both diseases, ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章,评审

    doi:10.1093/jmcb/mjq058

    authors: Du L,Pertsemlidis A

    更新日期:2011-06-01 00:00:00

  • Molecular suicide notes: last call from apoptosing cells.

    abstract::Dying cells actively attract professional phagocytes by sending out soluble 'find-me' signals. The characterization and identification of the corresponding molecular entities have revealed that 'find-me' signals come in different flavors, including peptides, proteins, lipids and complex structures, such as apoptotic m...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjp045

    authors: Peter C,Wesselborg S,Lauber K

    更新日期:2010-04-01 00:00:00

  • NSD3S stabilizes MYC through hindering its interaction with FBXW7.

    abstract::The MYC transcription factor plays a key role in cell growth control. Enhanced MYC protein stability has been found to promote tumorigenesis. Thus, understanding how MYC stability is controlled may have significant implications for revealing MYC-driven growth regulatory mechanisms in physiological and pathological pro...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjz098

    authors: Gonzalez-Pecchi V,Kwan AK,Doyle S,Ivanov AA,Du Y,Fu H

    更新日期:2020-07-03 00:00:00

  • NKT cell self-reactivity: evolutionary master key of immune homeostasis?

    abstract::Complex immune responses have evolved to protect multicellular organisms against the invasion of pathogens. This has exerted strong developmental pressure for specialized functions that can also limit damage to self-tissue. Two arms of immunity, the innate and adaptive immune systems, have evolved for quick, non-speci...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章,评审

    doi:10.1093/jmcb/mjr035

    authors: Issazadeh-Navikas S

    更新日期:2012-04-01 00:00:00

  • New march towards the regeneration of sensation and cognition: hear more, see more and learn more.

    abstract::As many human sensory and cognitive diseases are caused by irreversible damage or loss of certain types of neurons, methodologies aimed at replacement of lost neurons are key to restore lost sensation. Recent advances in generation of ear-cell progenitors, optic-cup structures and cortical neurons from human embryonic...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章,评审

    doi:10.1093/jmcb/mjs060

    authors: Zhang K,Yi F,Liu GH,Belmonte JC

    更新日期:2013-04-01 00:00:00

  • The crossregulation between ERK and PI3K signaling pathways determines the tumoricidal efficacy of MEK inhibitor.

    abstract::MEK inhibitor has been highlighted as a promising anti-tumor drug but its effect has been reported as varying over a wide range depending on patho-physiological conditions. In this study, we employed a systems approach by combining biochemical experimentation with in silico simulations to investigate the resistance me...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjs021

    authors: Won JK,Yang HW,Shin SY,Lee JH,Heo WD,Cho KH

    更新日期:2012-06-01 00:00:00

  • Extracellular vesicle-mediated transfer of donor genomic DNA to recipient cells is a novel mechanism for genetic influence between cells.

    abstract::Extracellular vesicles (EVs) carry signals within or at their limiting membranes, providing a mechanism by which cells can exchange more complex information than what was previously thought. In addition to mRNAs and microRNAs, there are DNA fragments in EVs. Solexa sequencing indicated the presence of at least 16434 g...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjt011

    authors: Cai J,Han Y,Ren H,Chen C,He D,Zhou L,Eisner GM,Asico LD,Jose PA,Zeng C

    更新日期:2013-08-01 00:00:00

  • Adaptor protein APPL1 links neuronal activity to chromatin remodeling in cultured hippocampal neurons.

    abstract::Local signaling events at synapses or axon terminals are communicated to the nucleus to elicit transcriptional responses, and thereby translate information about the external environment into internal neuronal representations. This retrograde signaling is critical to dendritic growth, synapse development, and neuronal...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjaa058

    authors: Wu Y,Lv X,Wang H,Qian K,Ding J,Wang J,Hua S,Sun T,Zhou Y,Yu L,Qiu S

    更新日期:2020-10-26 00:00:00

  • When Shigella tells the cell to hang on.

    abstract::OspE, a Shigella type III effector binds to integrin-like kinase and enhances cell adhesion to better disseminate and colonize the intestinal epithelium. Because of the existence of OspE orthologues in other enteropathogens such as enteropathogenic Escherichia coli or Salmonella sp., maintenance of cell adhesion appea...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjp013

    authors: Van Nhieu GT,Guignot J

    更新日期:2009-12-01 00:00:00

  • Global epigenetic changes during somatic cell reprogramming to iPS cells.

    abstract::Embryonic stem cells (ESCs) exhibit unique chromatin features, including a permissive transcriptional program and an open, decondensed chromatin state. Induced pluripotent stem cells (iPSCs), which are very similar to ESCs, hold great promise for therapy and basic research. However, the mechanisms by which reprogrammi...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjr028

    authors: Mattout A,Biran A,Meshorer E

    更新日期:2011-12-01 00:00:00

  • E-selectin ligand complexes adopt an extended high-affinity conformation.

    abstract::E-selectin is a cell-adhesion molecule of the vascular endothelium that promotes essential leukocyte rolling in the early inflammatory response by binding to glycoproteins containing the tetrasaccharide sialyl Lewis(x) (sLe(x)). Efficient leukocyte recruitment under vascular flow conditions depends on an increased lif...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjv046

    authors: Preston RC,Jakob RP,Binder FP,Sager CP,Ernst B,Maier T

    更新日期:2016-02-01 00:00:00

  • The role of RAD9 in tumorigenesis.

    abstract::RAD9 regulates multiple cellular processes that influence genomic integrity, and for at least some of its functions the protein acts as part of a heterotrimeric complex bound to HUS1 and RAD1 proteins. RAD9 participates in DNA repair, including base excision repair, homologous recombination repair and mismatch repair,...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章,评审

    doi:10.1093/jmcb/mjq039

    authors: Lieberman HB,Bernstock JD,Broustas CG,Hopkins KM,Leloup C,Zhu A

    更新日期:2011-02-01 00:00:00

  • Egg CD9 protein tides correlated with sperm oscillations tune the gamete fusion ability in mammal.

    abstract::Mammalian fertilization involves membrane events-adhesion, fusion, sperm engulfment, membrane block to polyspermy-whose causes remain largely unknown. Recently, specific oscillations of the sperm in contact with the egg were shown to be necessary for fusion. Using a microfluidic chip to impose the venue for the encoun...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjy005

    authors: Ravaux B,Favier S,Perez E,Gourier C

    更新日期:2018-12-01 00:00:00

  • Oxygen sufficiency controls TOP mRNA translation via the TSC-Rheb-mTOR pathway in a 4E-BP-independent manner.

    abstract::Cells encountering hypoxic stress conserve resources and energy by downregulating the protein synthesis. Here we demonstrate that one mechanism in this response is the translational repression of TOP mRNAs that encode components of the translational apparatus. This mode of regulation involves TSC and Rheb, as knockout...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mju008

    authors: Miloslavski R,Cohen E,Avraham A,Iluz Y,Hayouka Z,Kasir J,Mudhasani R,Jones SN,Cybulski N,Rüegg MA,Larsson O,Gandin V,Rajakumar A,Topisirovic I,Meyuhas O

    更新日期:2014-06-01 00:00:00

  • Myeloid adrenergic signaling via CaMKII forms a feedforward loop of catecholamine biosynthesis.

    abstract::Type 2 immune response has been shown to facilitate cold-induced thermogenesis and browning of white fat. However, whether alternatively activated macrophages produce catecholamine and substantially promote adaptive thermogenesis in adipose tissue remains controversial. Here, we show that tyrosine hydroxylase (TyrH), ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjx046

    authors: Luo Y,Liu B,Yang X,Ma X,Zhang X,Bragin DE,Yang XO,Huang W,Liu M

    更新日期:2017-10-01 00:00:00

  • Deregulation of tumor suppressive ASXL1-PTEN/AKT axis in myeloid malignancies.

    abstract::Mutations of epigenetic regulators are pervasive in human tumors. ASXL1 is frequently mutated in myeloid malignancies. We previously found that ASXL1 forms together with BAP1 a complex that can deubiquitinylate mono-ubiquitinylated lysine 119 on histone H2A (H2AK119ub1), a Polycomb repressive mark. However, a complete...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjaa011

    authors: Cao L,Xia X,Kong Y,Jia F,Yuan B,Li R,Li Q,Wang Y,Cui M,Dai Z,Zheng H,Christensen J,Zhou Y,Wu X

    更新日期:2020-09-01 00:00:00

  • Cancer-associated adipocyte-derived G-CSF promotes breast cancer malignancy via Stat3 signaling.

    abstract::Adipocyte is the most predominant cell type in the tumor microenvironment of breast cancer and plays a pivotal role in cancer progression, yet the underlying mechanisms and functional mediators remain elusive. We isolated primary preadipocytes from mammary fat pads of human breast cancer patients and generated mature ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjaa016

    authors: Liu L,Wu Y,Zhang C,Zhou C,Li Y,Zeng Y,Zhang C,Li R,Luo D,Wang L,Zhang L,Tu S,Deng H,Luo S,Chen YG,Xiong X,Yan X

    更新日期:2020-09-01 00:00:00

  • AFF3-DNA methylation interplay in maintaining the mono-allelic expression pattern of XIST in terminally differentiated cells.

    abstract::X chromosome inactivation and genomic imprinting are two classic epigenetic regulatory processes that cause mono-allelic gene expression. In female mammals, mono-allelic expression of the long non-coding RNA gene X-inactive specific transcript (XIST) is essential for initiation of X chromosome inactivation upon differ...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjy074

    authors: Zhang Y,Wang C,Liu X,Yang Q,Ji H,Yang M,Xu M,Zhou Y,Xie W,Luo Z,Lin C

    更新日期:2019-09-19 00:00:00

  • microRNA-129-5p, a c-Myc negative target, affects hepatocellular carcinoma progression by blocking the Warburg effect.

    abstract::Deregulation of microRNAs (miRNAs) and c-Myc (Myc) contributes to hepatocellular carcinoma (HCC) progression, but how miRNAs and Myc regulate each other in hepatocarcinogenesis is still poorly understood. Using a functional screen, we identified miR-129-5p as a miRNA that inhibits HCC cell growth. miR-129-5p targets t...

    journal_title:Journal of molecular cell biology

    pub_type: 杂志文章

    doi:10.1093/jmcb/mjw010

    authors: Han H,Li W,Shen H,Zhang J,Zhu Y,Li Y

    更新日期:2016-10-01 00:00:00