当前位置： SCI文献检索 > Nature Communications期刊下所有文献 > A gatekeeper helix determines the substrate specificity of Sjögren-Larsson Syndrome enzyme fatty aldehyde dehydrogenase.

A gatekeeper helix determines the substrate specificity of Sjögren-Larsson Syndrome enzyme fatty aldehyde dehydrogenase.

Abstract：

:Mutations in the gene coding for membrane-bound fatty aldehyde dehydrogenase (FALDH) lead to toxic accumulation of lipid species and development of the Sjögren-Larsson Syndrome (SLS), a rare disorder characterized by skin defects and mental retardation. Here, we present the crystallographic structure of human FALDH, the first model of a membrane-associated aldehyde dehydrogenase. The dimeric FALDH displays a previously unrecognized element in its C-terminal region, a 'gatekeeper' helix, which extends over the adjacent subunit, controlling the access to the substrate cavity and helping orientate both substrate cavities towards the membrane surface for efficient substrate transit between membranes and catalytic site. Activity assays demonstrate that the gatekeeper helix is important for directing the substrate specificity of FALDH towards long-chain fatty aldehydes. The gatekeeper feature is conserved across membrane-associated aldehyde dehydrogenases. Finally, we provide insight into the previously elusive molecular basis of SLS-causing mutations.

journal_name

Nat Commun

journal_title

Nature communications

authors

Keller MA,Zander U,Fuchs JE,Kreutz C,Watschinger K,Mueller T,Golderer G,Liedl KR,Ralser M,Kräutler B,Werner ER,Marquez JA

doi

10.1038/ncomms5439

subject

Has Abstract

pub_date

2014-07-22 00:00:00

pages

4439

issn

2041-1723

pii

ncomms5439

journal_volume

5

pub_type

杂志文章

相关文献

Nature Communications文献大全
  • Giant enhancement in vertical conductivity of stacked CVD graphene sheets by self-assembled molecular layers.

    abstract：:Layer-by-layer-stacked chemical vapour deposition (CVD) graphene films find applications as transparent and conductive electrodes in solar cells, organic light-emitting diodes and touch panels. Common to lamellar-type systems with anisotropic electron delocalization, the plane-to-plane (vertical) conductivity in such ...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms6461

    authors： Liu Y,Yuan L,Yang M,Zheng Y,Li L,Gao L,Nerngchamnong N,Nai CT,Sangeeth CS,Feng YP,Nijhuis CA,Loh KP

    更新日期：2014-11-20 00:00:00

  • The neuropeptide NLP-22 regulates a sleep-like state in Caenorhabditis elegans.

    abstract：:Neuropeptides have central roles in the regulation of homoeostatic behaviours such as sleep and feeding. Caenorhabditis elegans displays sleep-like quiescence of locomotion and feeding during a larval transition stage called lethargus and feeds during active larval and adult stages. Here we show that the neuropeptide ...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms3846

    authors： Nelson MD,Trojanowski NF,George-Raizen JB,Smith CJ,Yu CC,Fang-Yen C,Raizen DM

    更新日期：2013-01-01 00:00:00

  • Ultra-high on-chip optical gain in erbium-based hybrid slot waveguides.

    abstract：:Efficient and reliable on-chip optical amplifiers and light sources would enable versatile integration of various active functionalities on the silicon platform. Although lasing on silicon has been demonstrated with semiconductors by using methods such as wafer bonding or molecular beam epitaxy, cost-effective mass pr...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-019-08369-w

    authors： Rönn J,Zhang W,Autere A,Leroux X,Pakarinen L,Alonso-Ramos C,Säynätjoki A,Lipsanen H,Vivien L,Cassan E,Sun Z

    更新日期：2019-01-25 00:00:00

  • Prothymosin α overexpression contributes to the development of pulmonary emphysema.

    abstract：:Emphysema is one of the disease conditions that comprise chronic obstructive pulmonary disease. Prothymosin α transgenic mice exhibit an emphysema phenotype, but the pathophysiological role of prothymosin α in emphysema remains unclear. Here we show that prothymosin α contributes to the pathogenesis of emphysema by in...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms2906

    authors： Su BH,Tseng YL,Shieh GS,Chen YC,Shiang YC,Wu P,Li KJ,Yen TH,Shiau AL,Wu CL

    更新日期：2013-01-01 00:00:00

  • Synapsin II desynchronizes neurotransmitter release at inhibitory synapses by interacting with presynaptic calcium channels.

    abstract：:In the central nervous system, most synapses show a fast mode of neurotransmitter release known as synchronous release followed by a phase of asynchronous release, which extends over tens of milliseconds to seconds. Synapsin II (SYN2) is a member of the multigene synapsin family (SYN1/2/3) of synaptic vesicle phosphop...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms2515

    authors： Medrihan L,Cesca F,Raimondi A,Lignani G,Baldelli P,Benfenati F

    更新日期：2013-01-01 00:00:00

  • Long-range exciton transport and slow annihilation in two-dimensional hybrid perovskites.

    abstract：:Two-dimensional hybrid organic-inorganic perovskites with strongly bound excitons and tunable structures are desirable for optoelectronic applications. Exciton transport and annihilation are two key processes in determining device efficiencies; however, a thorough understanding of these processes is hindered by that a...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-14403-z

    authors： Deng S,Shi E,Yuan L,Jin L,Dou L,Huang L

    更新日期：2020-01-31 00:00:00

  • Hair cell force generation does not amplify or tune vibrations within the chicken basilar papilla.

    abstract：:Frequency tuning within the auditory papilla of most non-mammalian species is electrical, deriving from ion-channel resonance within their sensory hair cells. In contrast, tuning within the mammalian cochlea is mechanical, stemming from active mechanisms within outer hair cells that amplify the basilar membrane travel...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms13133

    authors： Xia A,Liu X,Raphael PD,Applegate BE,Oghalai JS

    更新日期：2016-10-31 00:00:00

  • Efficient assembly of nanopore reads via highly accurate and intact error correction.

    abstract：:Long nanopore reads are advantageous in de novo genome assembly. However, nanopore reads usually have broad error distribution and high-error-rate subsequences. Existing error correction tools cannot correct nanopore reads efficiently and effectively. Most methods trim high-error-rate subsequences during error correct...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-20236-7

    authors： Chen Y,Nie F,Xie SQ,Zheng YF,Dai Q,Bray T,Wang YX,Xing JF,Huang ZJ,Wang DP,He LJ,Luo F,Wang JX,Liu YZ,Xiao CL

    更新日期：2021-01-04 00:00:00

  • The nasal methylome as a biomarker of asthma and airway inflammation in children.

    abstract：:The nasal cellular epigenome may serve as biomarker of airway disease and environmental response. Here we collect nasal swabs from the anterior nares of 547 children (mean-age 12.9 y), and measure DNA methylation (DNAm) with the Infinium MethylationEPIC BeadChip. We perform nasal Epigenome-Wide Association analyses (E...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-019-11058-3

    authors： Cardenas A,Sordillo JE,Rifas-Shiman SL,Chung W,Liang L,Coull BA,Hivert MF,Lai PS,Forno E,Celedón JC,Litonjua AA,Brennan KJ,DeMeo DL,Baccarelli AA,Oken E,Gold DR

    更新日期：2019-07-12 00:00:00

  • Radiation-induced DNA damage and repair effects on 3D genome organization.

    abstract：:The three-dimensional structure of chromosomes plays an important role in gene expression regulation and also influences the repair of radiation-induced DNA damage. Genomic aberrations that disrupt chromosome spatial domains can lead to diseases including cancer, but how the 3D genome structure responds to DNA damage ...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-20047-w

    authors： Sanders JT,Freeman TF,Xu Y,Golloshi R,Stallard MA,Hill AM,San Martin R,Balajee AS,McCord RP

    更新日期：2020-12-02 00:00:00

  • Structural heterogeneity of α-synuclein fibrils amplified from patient brain extracts.

    abstract：:Parkinson's disease (PD) and Multiple System Atrophy (MSA) are clinically distinctive diseases that feature a common neuropathological hallmark of aggregated α-synuclein. Little is known about how differences in α-synuclein aggregate structure affect disease phenotype. Here, we amplified α-synuclein aggregates from PD...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-019-13564-w

    authors： Strohäker T,Jung BC,Liou SH,Fernandez CO,Riedel D,Becker S,Halliday GM,Bennati M,Kim WS,Lee SJ,Zweckstetter M

    更新日期：2019-12-04 00:00:00

  • The FNIP co-chaperones decelerate the Hsp90 chaperone cycle and enhance drug binding.

    abstract：:Heat shock protein-90 (Hsp90) is an essential molecular chaperone in eukaryotes involved in maintaining the stability and activity of numerous signalling proteins, also known as clients. Hsp90 ATPase activity is essential for its chaperone function and it is regulated by co-chaperones. Here we show that the tumour sup...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms12037

    authors： Woodford MR,Dunn DM,Blanden AR,Capriotti D,Loiselle D,Prodromou C,Panaretou B,Hughes PF,Smith A,Ackerman W,Haystead TA,Loh SN,Bourboulia D,Schmidt LS,Marston Linehan W,Bratslavsky G,Mollapour M

    更新日期：2016-06-29 00:00:00

  • Coordinated force generation of skeletal myosins in myofilaments through motor coupling.

    abstract：:In contrast to processive molecular motors, skeletal myosins form a large motor ensemble for contraction of muscles against high loads. Despite numerous information on the molecular properties of skeletal myosin, its ensemble effects on collective force generation have not been rigorously clarified. Here we show 4 nm ...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms16036

    authors： Kaya M,Tani Y,Washio T,Hisada T,Higuchi H

    更新日期：2017-07-06 00:00:00

  • LncRNA DILA1 inhibits Cyclin D1 degradation and contributes to tamoxifen resistance in breast cancer.

    abstract：:Cyclin D1 is one of the most important oncoproteins that drives cancer cell proliferation and associates with tamoxifen resistance in breast cancer. Here, we identify a lncRNA, DILA1, which interacts with Cyclin D1 and is overexpressed in tamoxifen-resistant breast cancer cells. Mechanistically, DILA1 inhibits the pho...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-19349-w

    authors： Shi Q,Li Y,Li S,Jin L,Lai H,Wu Y,Cai Z,Zhu M,Li Q,Li Y,Wang J,Liu Y,Wu Z,Song E,Liu Q

    更新日期：2020-11-02 00:00:00

  • BK channel inactivation gates daytime excitability in the circadian clock.

    abstract：:Inactivation is an intrinsic property of several voltage-dependent ion channels, closing the conduction pathway during membrane depolarization and dynamically regulating neuronal activity. BK K(+) channels undergo N-type inactivation via their β2 subunit, but the physiological significance is not clear. Here, we repor...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms10837

    authors： Whitt JP,Montgomery JR,Meredith AL

    更新日期：2016-03-04 00:00:00

  • Imaging Coulomb islands in a quantum Hall interferometer.

    abstract：:In the quantum Hall regime, near integer filling factors, electrons should only be transmitted through spatially separated edge states. However, in mesoscopic systems, electronic transmission turns out to be more complex, giving rise to a large spectrum of magnetoresistance oscillations. To explain these observations,...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms1038

    authors： Hackens B,Martins F,Faniel S,Dutu CA,Sellier H,Huant S,Pala M,Desplanque L,Wallart X,Bayot V

    更新日期：2010-07-27 00:00:00

  • Erratum: Free-standing supramolecular hydrogel objects by reaction-diffusion.

    abstract：:This corrects the article DOI: 10.1038/ncomms15317. ...

    journal_title：Nature communications

    pub_type： 杂志文章,已发布勘误

    doi：10.1038/ncomms16128

    authors： Lovrak M,Hendriksen WEJ,Maity C,Mytnyk S,van Steijn V,Eelkema R,van Esch JH

    更新日期：2017-06-30 00:00:00

  • Shuttling a single charge across a one-dimensional array of silicon quantum dots.

    abstract：:Significant advances have been made towards fault-tolerant operation of silicon spin qubits, with single qubit fidelities exceeding 99.9%, several demonstrations of two-qubit gates based on exchange coupling, and the achievement of coherent single spin-photon coupling. Coupling arbitrary pairs of spatially separated q...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-019-08970-z

    authors： Mills AR,Zajac DM,Gullans MJ,Schupp FJ,Hazard TM,Petta JR

    更新日期：2019-03-05 00:00:00

  • Atrx inactivation drives disease-defining phenotypes in glioma cells of origin through global epigenomic remodeling.

    abstract：:Mutational inactivation of the SWI/SNF chromatin regulator ATRX occurs frequently in gliomas, the most common primary brain tumors. Whether and how ATRX deficiency promotes oncogenesis by epigenomic dysregulation remains unclear, despite its recent implication in both genomic instability and telomere dysfunction. Here...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-018-03476-6

    authors： Danussi C,Bose P,Parthasarathy PT,Silberman PC,Van Arnam JS,Vitucci M,Tang OY,Heguy A,Wang Y,Chan TA,Riggins GJ,Sulman EP,Lang F,Creighton CJ,Deneen B,Miller CR,Picketts DJ,Kannan K,Huse JT

    更新日期：2018-03-13 00:00:00

  • Site-selective photoemission from delocalized valence shells induced by molecular rotation.

    abstract：:Due to the generally delocalized nature of molecular valence orbitals, valence-shell spectroscopies do not usually allow to specifically target a selected atom in a molecule. However, in X-ray electron spectroscopy, the photoelectron momentum is large and the recoil angular momentum transferred to the molecule is larg...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms4816

    authors： Miron C,Miao Q,Nicolas C,Bozek JD,Andrałojć W,Patanen M,Simões G,Travnikova O,Ågren H,Gel'mukhanov F

    更新日期：2014-05-09 00:00:00

  • Amplification and generation of ultra-intense twisted laser pulses via stimulated Raman scattering.

    abstract：:Twisted Laguerre-Gaussian lasers, with orbital angular momentum and characterized by doughnut-shaped intensity profiles, provide a transformative set of tools and research directions in a growing range of fields and applications, from super-resolution microcopy and ultra-fast optical communications to quantum computin...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms10371

    authors： Vieira J,Trines RM,Alves EP,Fonseca RA,Mendonça JT,Bingham R,Norreys P,Silva LO

    更新日期：2016-01-28 00:00:00

  • Repression of RNA polymerase by the archaeo-viral regulator ORF145/RIP.

    abstract：:Little is known about how archaeal viruses perturb the transcription machinery of their hosts. Here we provide the first example of an archaeo-viral transcription factor that directly targets the host RNA polymerase (RNAP) and efficiently represses its activity. ORF145 from the temperate Acidianus two-tailed virus (AT...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms13595

    authors： Sheppard C,Blombach F,Belsom A,Schulz S,Daviter T,Smollett K,Mahieu E,Erdmann S,Tinnefeld P,Garrett R,Grohmann D,Rappsilber J,Werner F

    更新日期：2016-11-24 00:00:00

  • Systemic nanoparticle delivery of CRISPR-Cas9 ribonucleoproteins for effective tissue specific genome editing.

    abstract：:CRISPR-Cas9 has emerged as a powerful technology that relies on Cas9/sgRNA ribonucleoprotein complexes (RNPs) to target and edit DNA. However, many therapeutic targets cannot currently be accessed due to the lack of carriers that can deliver RNPs systemically. Here, we report a generalizable methodology that allows en...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-17029-3

    authors： Wei T,Cheng Q,Min YL,Olson EN,Siegwart DJ

    更新日期：2020-06-26 00:00:00

  • Integrated multilayer stretchable printed circuit boards paving the way for deformable active matrix.

    abstract：:Conventional rigid electronic systems use a number of metallization layers to route all necessary connections to and from isolated surface mount devices using well-established printed circuit board technology. In contrast, present solutions to prepare stretchable electronic systems are typically confined to a single s...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-019-12870-7

    authors： Biswas S,Schoeberl A,Hao Y,Reiprich J,Stauden T,Pezoldt J,Jacobs HO

    更新日期：2019-10-28 00:00:00

  • Profiling immunoglobulin repertoires across multiple human tissues using RNA sequencing.

    abstract：:Profiling immunoglobulin (Ig) receptor repertoires with specialized assays can be cost-ineffective and time-consuming. Here we report ImReP, a computational method for rapid and accurate profiling of the Ig repertoire, including the complementary-determining region 3 (CDR3), using regular RNA sequencing data such as t...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-16857-7

    authors： Mandric I,Rotman J,Yang HT,Strauli N,Montoya DJ,Van Der Wey W,Ronas JR,Statz B,Yao D,Petrova V,Zelikovsky A,Spreafico R,Shifman S,Zaitlen N,Rossetti M,Ansel KM,Eskin E,Mangul S

    更新日期：2020-06-19 00:00:00

  • Observation of an unpaired photonic Dirac point.

    abstract：:At photonic Dirac points, electromagnetic waves are governed by the same equations as two-component massless relativistic fermions. However, photonic Dirac points are known to occur in pairs in "photonic graphene" and other similar photonic crystals, which necessitates special precautions to excite only one valley sta...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-15801-z

    authors： Liu GG,Zhou P,Yang Y,Xue H,Ren X,Lin X,Sun HX,Bi L,Chong Y,Zhang B

    更新日期：2020-04-20 00:00:00

  • Clonal hematopoiesis is associated with adverse outcomes in multiple myeloma patients undergoing transplant.

    abstract：:Multiple myeloma (MM) is a plasma-cell neoplasm that is treated with high-dose chemotherapy, autologous stem cell transplant (ASCT) and long-term immunomodulatory drug (IMiD) maintenance. The presence of somatic mutations in the peripheral blood is termed clonal hematopoiesis of indeterminate potential (CHIP) and is a...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-16805-5

    authors： Mouhieddine TH,Sperling AS,Redd R,Park J,Leventhal M,Gibson CJ,Manier S,Nassar AH,Capelletti M,Huynh D,Bustoros M,Sklavenitis-Pistofidis R,Tahri S,Hornburg K,Dumke H,Itani MM,Boehner CJ,Liu CJ,AlDubayan SH,Reardon B

    更新日期：2020-06-12 00:00:00

  • Tug-of-war between actomyosin-driven antagonistic forces determines the positioning symmetry in cell-sized confinement.

    abstract：:Symmetric or asymmetric positioning of intracellular structures including the nucleus and mitotic spindle steers various biological processes such as cell migration, division, and embryogenesis. In typical animal cells, both a sparse actomyosin meshwork in the cytoplasm and a dense actomyosin cortex underneath the cel...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-020-16677-9

    authors： Sakamoto R,Tanabe M,Hiraiwa T,Suzuki K,Ishiwata S,Maeda YT,Miyazaki M

    更新日期：2020-06-15 00:00:00

  • An unusual endo-selective C-H hydroarylationof norbornene by the Rh(I)-catalyzed reactionof benzamides.

    abstract：:Hydroarylation is an environmentally attractive strategy which incorporates all of the atoms contained in the substrates into the desired products. Almost all the hydroarylations of norbornene reported to date involve an exo-selective reaction. Here we show the endo-selective hydroarylation of norbornene in the Rh(I)-...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/s41467-017-01531-2

    authors： Shibata K,Natsui S,Tobisu M,Fukumoto Y,Chatani N

    更新日期：2017-11-13 00:00:00

  • Grain rotation mediated by grain boundary dislocations in nanocrystalline platinum.

    abstract：:Grain rotation is a well-known phenomenon during high (homologous) temperature deformation and recrystallization of polycrystalline materials. In recent years, grain rotation has also been proposed as a plasticity mechanism at low temperatures (for example, room temperature for metals), especially for nanocrystalline ...

    journal_title：Nature communications

    pub_type： 杂志文章

    doi：10.1038/ncomms5402

    authors： Wang L,Teng J,Liu P,Hirata A,Ma E,Zhang Z,Chen M,Han X

    更新日期：2014-07-17 00:00:00