Proton magnetic resonance spectroscopy of Sjögren-Larsson syndrome heterozygotes.

Abstract:

:A deficit of fatty alcohol:NAD+ oxidoreductase complex (FAO) activity has been detected in patients with the Sjögren-Larsson syndrome (SLS). A moderate decrease in FAO activity has also been reported in heterozygote SLS subjects. Abnormal peaks were detected with proton magnetic resonance spectroscopy (1H-MRS) in homozygote SLS subjects. The purpose of this study was to examine whether 1H-MRS can be used to detect metabolic and/or pathological abnormalities in heterozygote SLS subjects. Four SLS heterozygotes were examined using 1H-MRS. A moderate decrease in FAO activity was demonstrated in two of the four heterozygotes. Abnormal peaks were detected at 0.9 ppm in the spectrum from cerebral hemispheres of every heterozygote. 1H-MRS was able to detect an abnormal accumulation of fatty alcohols and lipids, which is expected to increase due to an decrease in FAO activity or dysmyelination in heterozygote SLS subjects. Thus, 1H-MRS is suggested to be a powerful tool in the screening of SLS heterozygotes.

journal_name

Magn Reson Med

authors

Kaminaga T,Mano T,Ono J,Kusuoka H,Nakamura H,Nishimura T

doi

10.1002/mrm.1146

subject

Has Abstract

pub_date

2001-06-01 00:00:00

pages

1112-5

issue

6

eissn

0740-3194

issn

1522-2594

pii

10.1002/mrm.1146

journal_volume

45

pub_type

杂志文章
  • Ultrahigh field single-refocused diffusion weighted imaging using a matched-phase adiabatic spin echo (MASE).

    abstract:PURPOSE:To improve ultrahigh field diffusion-weighted imaging (DWI) in the presence of inhomogeneous transmit B1 field by designing a novel semi-adiabatic single-refocused DWI technique. METHODS:A 180° slice-selective, adiabatic radiofrequency (RF) pulse of 4 ms duration was designed using the adiabatic Shinnar-Le Rou...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.25790

    authors: Dyvorne H,O'Halloran R,Balchandani P

    更新日期:2016-05-01 00:00:00

  • Bayesian multipoint velocity encoding for concurrent flow and turbulence mapping.

    abstract::An approach to efficiently measure three-dimensional velocity vector fields and turbulent kinetic energy of blood flow is presented. Multipoint phase-contrast imaging is used in combination with Bayesian analysis to map both mean and fluctuating velocities over a large dynamic range and for practically relevant signal...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.24370

    authors: Binter C,Knobloch V,Manka R,Sigfridsson A,Kozerke S

    更新日期:2013-05-01 00:00:00

  • Kinetic modeling of hyperpolarized (13)C pyruvate metabolism in tumors using a measured arterial input function.

    abstract::Mathematical models are required to estimate kinetic parameters of [1-(13)C] pyruvate-lactate interconversion from magnetic resonance spectroscopy data. One- or two-way exchange models utilizing a hypothetical approximation to the true arterial input function (AIF), (e.g. an ideal 'box-car' function) have been used pr...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.24546

    authors: Kazan SM,Reynolds S,Kennerley A,Wholey E,Bluff JE,Berwick J,Cunningham VJ,Paley MN,Tozer GM

    更新日期:2013-10-01 00:00:00

  • Phase-sensitive inversion recovery for myocardial T1 mapping with motion correction and parametric fitting.

    abstract::The assessment of myocardial fibrosis and extracellular volume requires accurate estimation of myocardial T1 s. While image acquisition using the modified Look-Locker inversion recovery technique is clinically feasible for myocardial T1 mapping, respiratory motion can limit its applicability. Moreover, the conventiona...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.24385

    authors: Xue H,Greiser A,Zuehlsdorff S,Jolly MP,Guehring J,Arai AE,Kellman P

    更新日期:2013-05-01 00:00:00

  • Macromolecule and water magnetization exchange modeling in articular cartilage.

    abstract::Magnetization exchange effects between the matrix macromolecules (e. g., collagen and proteoglycan) and water were examined in normal, deuterated, and proteoglycan-depleted articular cartilage. Relaxation results (T(2), T(1rho), and T(1)) suggested that a four-site exchange scheme provided an accurate model for articu...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/1522-2594(200012)44:6<840::aid-mrm4>3.0.co

    authors: Lattanzio PJ,Marshall KW,Damyanovich AZ,Peemoeller H

    更新日期:2000-12-01 00:00:00

  • Quantifying tissue damage due to focused ultrasound heating observed by MRI.

    abstract::Focused ultrasound heating of ex vivo bovine kidney and liver was monitored using magnetic resonance imaging (MRI) to investigate the quantitative relationship between time-dependent temperature elevations and altered contrast in MR images due to thermal coagulation. Proton resonance frequency shift MR thermometry was...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/(sici)1522-2594(199902)41:2<321::aid-mrm16

    authors: Graham SJ,Chen L,Leitch M,Peters RD,Bronskill MJ,Foster FS,Henkelman RM,Plewes DB

    更新日期:1999-02-01 00:00:00

  • Diffusional kurtosis imaging: the quantification of non-gaussian water diffusion by means of magnetic resonance imaging.

    abstract::A magnetic resonance imaging method is presented for quantifying the degree to which water diffusion in biologic tissues is non-Gaussian. Since tissue structure is responsible for the deviation of water diffusion from the Gaussian behavior typically observed in homogeneous solutions, this method provides a specific me...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.20508

    authors: Jensen JH,Helpern JA,Ramani A,Lu H,Kaczynski K

    更新日期:2005-06-01 00:00:00

  • Accuracy, repeatability, and interplatform reproducibility of T1 quantification methods used for DCE-MRI: Results from a multicenter phantom study.

    abstract:PURPOSE:To determine the in vitro accuracy, test-retest repeatability, and interplatform reproducibility of T1 quantification protocols used for dynamic contrast-enhanced MRI at 1.5 and 3 T. METHODS:A T1 phantom with 14 samples was imaged at eight centers with a common inversion-recovery spin-echo (IR-SE) protocol and...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.26903

    authors: Bane O,Hectors SJ,Wagner M,Arlinghaus LL,Aryal MP,Cao Y,Chenevert TL,Fennessy F,Huang W,Hylton NM,Kalpathy-Cramer J,Keenan KE,Malyarenko DI,Mulkern RV,Newitt DC,Russek SE,Stupic KF,Tudorica A,Wilmes LJ,Yankeelov TE

    更新日期:2018-05-01 00:00:00

  • Noninvasive analysis of aldose reductase activities in rat testis: 3-FDG NMR spectroscopy and imaging.

    abstract::Noninvasive investigation of aldose reductase activities in rat testis was performed using 3-fluoro-3-deoxy-D-glucose (3-FDG) and nuclear magnetic resonance (NMR) spectroscopy/imaging. Quantitative determination of testis aldose reductase activities, expressed as the sorbitol index, showed a value similar to that of b...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.1910290417

    authors: Nakada T,Kwee IL

    更新日期:1993-04-01 00:00:00

  • Spin-echo MRI using pi/2 and pi hyperbolic secant pulses.

    abstract::Frequency-modulated (FM) pulses have practical advantages for spin-echo experiments, such as the ability to produce a broadband pi rotation, with an inhomogeneous radiofrequency (RF) field. However, such use leads to a nonlinear phase of the transverse magnetization, which is why FM pulses like the hyperbolic secant (...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.21822

    authors: Park JY,Garwood M

    更新日期:2009-01-01 00:00:00

  • Fast 3D ultrashort echo-time spiral projection imaging using golden-angle: A flexible protocol for in vivo mouse imaging at high magnetic field.

    abstract:PURPOSE:To develop a fast three-dimensional (3D) k-space encoding method based on spiral projection imaging (SPI) with an interleaved golden-angle approach and to validate this novel sequence on small animal models. METHODS:A disk-like trajectory, in which each disk contained spirals, was developed. The 3D encoding wa...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.26263

    authors: Castets CR,Lefrançois W,Wecker D,Ribot EJ,Trotier AJ,Thiaudière E,Franconi JM,Miraux S

    更新日期:2017-05-01 00:00:00

  • Comparison between 2D and 3D gradient-echo sequences for MRI of human lung ventilation with hyperpolarized 3He.

    abstract::Images of hyperpolarized 3He were acquired during breath-hold in four healthy volunteers with the use of an optimized 3D gradient-echo sequence. The images were compared with existing 2D gradient-echo methods. The average SNR from a 13-mm-thick slice in the peripheral lung was 1.4 times greater with 3D. In the airways...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.20164

    authors: Wild JM,Woodhouse N,Paley MN,Fichele S,Said Z,Kasuboski L,van Beek EJ

    更新日期:2004-09-01 00:00:00

  • In vivo imaging of insulin-secreting human pancreatic ductal cells using MRI reporter gene technique: A feasibility study.

    abstract:PURPOSE:The purpose of this study was to investigate the feasibility of in vivo imaging of human pancreatic ductal cells by OATP1B3 reporter gene under MRI. METHODS:A human cell line (PANC-1) derived from the pancreatic ductal epithelium was used in this study. After transduction of OATP1B3, the cellular physiological...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.27749

    authors: Wu MR,Hsiao JK,Liu HM,Huang YY,Tseng YJ,Chou PT,Weng TI,Yang CY

    更新日期:2019-08-01 00:00:00

  • Dysprosium-bearing red cells as potential transverse relaxation agents for MRI.

    abstract::The cytosol of intact human red blood cells was loaded with 28.1 +/- 3.4 mM of dysprosium DTPA-BMA using a hypoosmotic technique. When loaded cells were diluted with saline and control cells to give an average dysprosium concentration of 3.3 +/- 0.5 mM, the transverse relaxation rate constants R(*)(2) and R(2) increas...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.1122

    authors: Johnson KM,Tao JZ,Kennan RP,Gore JC

    更新日期:2001-05-01 00:00:00

  • Investigation of resolution effects using a specialized diffusion tensor phantom.

    abstract:PURPOSE:The clinical potential of the diffusion imaging-based analysis of fine brain structures such as fornix or cingulum is high due to the central role of these structures in psychiatric diseases. However, the quantification of diffusion parameters in fine structures is especially prone to partial volume effects (PV...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.24774

    authors: Bach M,Fritzsche KH,Stieltjes B,Laun FB

    更新日期:2014-03-01 00:00:00

  • Molecular imaging of angiogenic therapy in peripheral vascular disease with alphanubeta3-integrin-targeted nanoparticles.

    abstract::Noninvasive molecular imaging of angiogenesis could play a critical role in the clinical management of peripheral vascular disease patients. The alpha(nu)beta(3)-integrin, a well-established biomarker of neovascular proliferation, is an ideal target for molecular imaging of angiogenesis. This study investigates whethe...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.22447

    authors: Winter PM,Caruthers SD,Allen JS,Cai K,Williams TA,Lanza GM,Wickline SA

    更新日期:2010-08-01 00:00:00

  • Motion correction in fMRI via registration of individual slices into an anatomical volume.

    abstract::An automated retrospective image registration based on mutual information is adapted to a multislice functional magnetic resonance imaging (fMRI) acquisition protocol to provide accurate motion correction. Motion correction is performed by mapping each slice to an anatomic volume data set acquired in the same fMRI ses...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/(sici)1522-2594(199905)41:5<964::aid-mrm16

    authors: Kim B,Boes JL,Bland PH,Chenevert TL,Meyer CR

    更新日期:1999-05-01 00:00:00

  • Unaliasing by fourier-encoding the overlaps using the temporal dimension (UNFOLD), applied to cardiac imaging and fMRI.

    abstract::In several applications, MRI is used to monitor the time behavior of the signal in an organ of interest; e.g., signal evolution because of physiological motion, activation, or contrast-agent accumulation. Dynamic applications involve acquiring data in a k-t space, which contains both temporal and spatial information. ...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/(sici)1522-2594(199911)42:5<813::aid-mrm1>

    authors: Madore B,Glover GH,Pelc NJ

    更新日期:1999-11-01 00:00:00

  • Cardiac gating using scattering of an 8-channel parallel transmit coil at 7T.

    abstract:PURPOSE:To establish a cardiac signal from scattering matrix or scattering coefficient measurements made on a 7T 8-channel parallel transmit (pTx) system, and to evaluate its use for cardiac gating. METHODS:Measurements of the scattering matrix and scattering coefficients were acquired using a monitoring pulse sequenc...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.27038

    authors: Jaeschke SHF,Robson MD,Hess AT

    更新日期:2018-08-01 00:00:00

  • Characterization of white matter alterations in phenylketonuria by magnetic resonance relaxometry and diffusion tensor imaging.

    abstract::A multimodal MR study including relaxometry, diffusion tensor imaging (DTI), and MR spectroscopy was performed on patients with classical phenylketonuria (PKU) and matched controls, to improve our understanding of white matter (WM) lesions. Relaxometry yields information on myelin loss or malformation and may substant...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.21422

    authors: Vermathen P,Robert-Tissot L,Pietz J,Lutz T,Boesch C,Kreis R

    更新日期:2007-12-01 00:00:00

  • Direct reconstruction of MR images from data acquired on a non-Cartesian grid using an equal-phase-line algorithm.

    abstract::The equal-phase line (EPL) algorithm is proposed as a means of allowing rapid Fourier transform (FT) reconstruction of MR image data acquired on a non-Cartesian grid. The pixels on the image are grouped according to their positions. The pixels in a group have the same phase in the complex exponential function -exp[j2p...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.10165

    authors: Qian Y,Lin J,Jin D

    更新日期:2002-06-01 00:00:00

  • In vivo waveguide elastography: effects of neurodegeneration in patients with amyotrophic lateral sclerosis.

    abstract:PURPOSE:Waveguide elastography (WGE) combines magnetic resonance elastography (MRE), diffusion tensor imaging (DTI), and anisotropic inversions for a determination of the elastic properties of white matter. Previously, the method evaluated the anisotropic elastic properties of the corticospinal tracts (CSTs) of healthy...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.25067

    authors: Romano A,Guo J,Prokscha T,Meyer T,Hirsch S,Braun J,Sack I,Scheel M

    更新日期:2014-12-01 00:00:00

  • Optimum flip-angles for exciting NMR with uncertain T1 values.

    abstract::T1 is often ill-determined. This means that an Ernst angle excitation often cannot be precisely defined for the simple pulse and acquire experiment. Here, published 31P T1 values of metabolites in human muscle, liver, heart, and brain are archived, some new data on heart and brain added, and overall confidence interva...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.1910320120

    authors: Bottomley PA,Ouwerkerk R

    更新日期:1994-07-01 00:00:00

  • Proton MRI as a noninvasive tool to assess elastase-induced lung damage in spontaneously breathing rats.

    abstract::Elastase-induced changes in lung morphology and function were detected in spontaneously breathing rats using conventional proton MRI at 4.7 T. A single dose of porcine pancreatic elastase (75 U/100 g body weight) or vehicle (saline) was administered intratracheally (i.t.) to male Brown Norway (BN) rats. MRI fluid sign...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.21051

    authors: Quintana HK,Cannet C,Zurbruegg S,Blé FX,Fozard JR,Page CP,Beckmann N

    更新日期:2006-12-01 00:00:00

  • Optimization and validation of accelerated golden-angle radial sparse MRI reconstruction with self-calibrating GRAPPA operator gridding.

    abstract:PURPOSE:Golden-angle radial sparse parallel (GRASP) MRI reconstruction requires gridding and regridding to transform data between radial and Cartesian k-space. These operations are repeatedly performed in each iteration, which makes the reconstruction computationally demanding. This work aimed to accelerate GRASP recon...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.27030

    authors: Benkert T,Tian Y,Huang C,DiBella EVR,Chandarana H,Feng L

    更新日期:2018-07-01 00:00:00

  • The effects of intravoxel contrast agent diffusion on the analysis of DCE-MRI data in realistic tissue domains.

    abstract:PURPOSE:Quantitative evaluation of dynamic contrast enhanced MRI (DCE-MRI) allows for estimating perfusion, vessel permeability, and tissue volume fractions by fitting signal intensity curves to pharmacokinetic models. These compart mental models assume rapid equilibration of contrast agent within each voxel. However, ...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.26995

    authors: Woodall RT,Barnes SL,Hormuth DA 2nd,Sorace AG,Quarles CC,Yankeelov TE

    更新日期:2018-07-01 00:00:00

  • Modulation of the pharmacokinetics of macromolecular contrast material by avidin chase: MRI, optical, and inductively coupled plasma mass spectrometry tracking of triply labeled albumin.

    abstract::The goal of this work was to develop an MRI method for mapping the clearance of interstitial macromolecular plasma proteins after their extravasation from permeable blood vessels. To that end, a well-defined window of exposure to elevated blood levels was generated by inducing rapid clearance of macromolecular contras...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.10638

    authors: Dafni H,Gilead A,Nevo N,Eilam R,Harmelin A,Neeman M

    更新日期:2003-11-01 00:00:00

  • Accelerated cardiac magnetic resonance imaging in the mouse using an eight-channel array at 9.4 Tesla.

    abstract::MRI has become an important tool to noninvasively assess global and regional cardiac function, infarct size, or myocardial blood flow in surgically or genetically modified mouse models of human heart disease. Constraints on scan time due to sensitivity to general anesthesia in hemodynamically compromised mice frequent...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.22605

    authors: Schneider JE,Lanz T,Barnes H,Stork LA,Bohl S,Lygate CA,Ordidge RJ,Neubauer S

    更新日期:2011-01-01 00:00:00

  • Rapid in vivo musculoskeletal MR with parallel imaging at 7T.

    abstract::The purpose of this work was to implement autocalibrating GRAPPA-based parallel imaging (PI) for in vivo high-resolution (HR) MRI of cartilage and trabecular bone micro-architecture at 7T and to evaluate its performance based on comparison of MR-derived morphology metrics between accelerated and conventional images an...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.21455

    authors: Banerjee S,Krug R,Carballido-Gamio J,Kelley DA,Xu D,Vigneron DB,Majumdar S

    更新日期:2008-03-01 00:00:00

  • A three-dimensional variable-density spiral spatial-spectral RF pulse with rotated gradients.

    abstract::Three-dimensional spatial-spectral radiofrequency pulses using a stack-of-spirals trajectory can achieve two-dimensional spatial localization and one-dimensional spectral selection simultaneously. These pulses are useful, for example, in reduced field-of-view applications that also require frequency specificity such a...

    journal_title:Magnetic resonance in medicine

    pub_type: 杂志文章

    doi:10.1002/mrm.22209

    authors: Deng W,Stenger VA

    更新日期:2010-03-01 00:00:00