• KOL
    • Immune Tolerance
    • Merry B Passage
    • Merry B Passage: Influence Statistics

      Merry B Passage

      Merry B Passage

      Department of Pediatrics, Los Angeles Biomedical Research Institute at Harbor‐UCLA Medical Center, Torrance, CA, USA | Department of Pediatrics, Los Angeles Biomedical ...

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      Merry B Passage:Expert Impact

      Concepts for whichMerry B Passagehas direct influence:Immune tolerance,Enzyme replacement therapy,Human chromosome,Canine mucopolysaccharidosis,Brain disease,Spinal cord compression,Sulphate transport,Marker chromosomes.

      Merry B Passage:KOL impact

      Concepts related to the work of other authors for whichfor which Merry B Passage has influence:Mucopolysaccharidosis type,Enzyme replacement therapy,Lysosomal storage,Hurler syndrome,Uniparental disomy,Pompe disease,Patients mps.

      KOL Resume for Merry B Passage

      Year
      2015

      Department of Pediatrics, Los Angeles Biomedical Research Institute at Harbor‐UCLA Medical Center, Torrance, CA, USA

      2012

      Department of Pediatrics, Los Angeles Biomedical Research Institute at Harbor-UCLA, Torrance, CA, 90502, USA

      2011

      Division of Medical Genetics, Los Angeles Biomedical Research Institute at Harbor-UCLA, Torrance, CA

      2010

      Los Angeles Biomedical Research Institute at Harbor–UCLA Medical Center, and the Department of Pediatrics at Harbor-UCLA Medical Center, Torrance, CA 90502, USA.

      2009

      Los Angeles Biomedical Research Institute at Harbor, UCLA, 1124 W. Carson Street, E‐4, Torrance, CA, 90502, USA

      2008

      Division of Medical Genetics, LA Biomed at Harbor-UCLA, Torrance, CA, Torrance, CA, USA

      2007

      Division of Medical Genetics, Department of Pediatrics, LA Biomed at Harbor-UCLA, 1124 W. Carson Street, Building E-4, Torrance, CA 90502, USA

      2005

      Division of Medical Genetics, Department of Pediatrics, Harbor-UCLA Medical Center and Los Angeles Biomedical Research Institute, Torrance, CA, USA

      2004

      Division of Medical Genetics, Department of Pediatrics, Harbor-UCLA Research and Education Institute, Torrance CA, USA

      2001

      From the Department of Pediatrics, Division of Medical Genetics, Harbor–UCLA Medical Center, Torrance, Calif. (E.D.K., M.P., B.I., J.P., R.D., I.W., R.H.)

      1998

      Division of Medical Genetics, E4, Department of Pediatrics, Harbor-UCLA Medical Center, 1124 W. Carson St., Torrance, CA 90502, U.S.A

      1997

      Division of Medical Genetics, Department of Pediatrics, Harbor-UCLA Medical Center, 1124 West Carson Street, Torrance, CA 90502, U.S.A

      1996

      Division of Medical Genetics, Harbor-UCLA Medical Center, 90509, Torrance, CA, USA

      1994

      Department of Pediatrics, Harbor-UCLA Medical Center, Torrance 90502.

      1993

      Division of Medical Genetics, Harbor-UCLA Medical Center, 90509, Torrance, California

      1992

      Department of Pediatrics, UCLA School of Medicine, Harbor-UCLA Medical Center, 90509, Torrance, California

      1989

      Division of Medical Genetics, Department of Pediatrics, Harbor-UCLA Medical Center, 90509, Torrance, California

      1985

      Division of Medical Genetics, Harbor‐UCLA Medical Center, Torrance, California

      1981

      Division of Medical Genetics, Harbor-UCLA Medical Center, Torrance, and, Division of Medical Genetics, UCLA School of Medicine, Los Angeles, Calif.

      1980

      Department of Zoology, Arizona State University, 85281, Tempe, Arizona, USA

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      Sample of concepts for which Merry B Passage is among the top experts in the world.
      Concept World rank
      hexosaminidase 30 #1
      lysosomal sulphate carrier #1
      272±123 pmol csf #1
      rhidu #1
      meningeal gag #1
      brain pgag #1
      lysosomal sulphate uptake #1
      atp sulphate uptake #1
      iduronidase rhidu #1
      tolerance immunoglobulin iduronidase #1
      sulphydryl groups role #1
      pmol unit #1
      monothiol binding reagents #1
      lysosomal sulphate #1
      role sulphate transport #1
      gag pgag #1
      sulphate carrier #1
      intrathecal enzyme #1
      pmolperunit beta #1
      pgag storage #1
      mps intrathecal enzyme #1
      pgag mps #1
      sulphate transport atp #1
      lysosomal sulphate transport #1
      sulphate uptake requirement #1
      cortical brain pgag #1
      mps adult #1
      rhidu mps #1
      vmax sulfhydryl reagents #1
      lysosomal sulfate transport #1
      tissue gag accumulation #1
      intrathecal recombinant #1
      pathologic gag #1
      intrathecal enzyme therapy #1
      pmolperunit #1
      spinal mucopolysaccharidosis mps #1
      titer alters #1
      pgag brain #1
      atp sulphate #1
      cerebrospinal fluid pgag #1
      iduronidase levels #1
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      Prominent publications by Merry B Passage

      KOL-Index: 11303

      Enzyme replacement therapy (ERT) has been developed for several lysosomal storage disorders, including mucopolysaccharidosis I (MPS I), and is effective at reducing lysosomal storage in many tissues and in ameliorating clinical disease. However, intravenous ERT does not adequately treat storage disease in the central nervous system (CNS), presumably due to effects of the blood-brain barrier on enzyme distribution. To circumvent this barrier, we studied whether intrathecal (IT) ...

      Known for Lysosomal Storage | Canine Model | Enzyme Replacement Therapy | Mucopolysaccharidosis Mps | Normal Levels
      KOL-Index: 9674

      Enzyme replacement therapy (ERT) with intravenous recombinant human alpha-l-iduronidase (IV rhIDU) is a treatment for patients with mucopolysaccharidosis I (MPS I). Spinal cord compression develops in MPS I patients due in part to dural and leptomeningeal thickening from accumulated glycosaminoglycans (GAG). We tested long-term and every 3-month intrathecal (IT) and weekly IV rhIDU in MPS I dogs age 12-15months (Adult) and MPS I pups age 2-23days (Early) to determine whether spinal cord ...

      Known for Spinal Cord | Replacement Therapy | Mps Patients | Mucopolysaccharidosis Type | Gag Storage
      KOL-Index: 9595

      Mucopolysaccharidoses (MPSs) are lysosomal storage diseases caused by a deficit in the enzymes needed for glycosaminoglycan (GAG) degradation. Enzyme replacement therapy with recombinant human alpha-L-iduronidase successfully reduces lysosomal storage in canines and humans with iduronidase-deficient MPS I, but therapy usually also induces antibodies specific for the recombinant enzyme that could reduce its efficacy. To understand the potential impact of alpha-L-iduronidase-specific ...

      Known for Immune Tolerance | Enzyme Replacement Therapy | Lysosomal Storage | Higher Dose | Canine Mucopolysaccharidosis
      KOL-Index: 8465

      Treatment of brain disease with recombinant proteins is difficult due to the blood-brain barrier. As an alternative to direct injections into the brain, we studied whether application of high concentrations of therapeutic enzymes via intrathecal (IT) injections could successfully drive uptake across the ependyma to treat brain disease. We studied IT enzyme replacement therapy with recombinant human iduronidase (rhIDU) in canine mucopolysaccharidosis I (MPS I, Hurler syndrome), a ...

      Known for Brain Disease | Cerebrospinal Fluid | Enzyme Replacement Therapy | Spinal Meninges | Successful Treatment
      KOL-Index: 8424

      BACKGROUND: Mucopolysaccharidosis I is a lysosomal storage disease caused by a deficiency of the enzyme alpha-L-iduronidase. We evaluated the effect of enzyme-replacement therapy with recombinant human alpha-L-iduronidase in patients with this disorder.

      METHODS: We treated 10 patients with mucopolysaccharidosis I (age, 5 to 22 years) with recombinant human alpha-L-iduronidase at a dose of 125,000 U per kilogram of body weight given intravenously once weekly for 52 weeks. The patients ...

      Known for Patients Mucopolysaccharidosis | 52 Weeks | Alpha Iduronidase | Replacement Therapy | Range Motion
      KOL-Index: 8173

      A 9-year-old mentally retarded girl with multiple congenital anomalies was found to carry a balanced 13/14 Robertsonian translocation [45,XX,t(13q14q)] which was also present in her father. Her mother carried a balanced reciprocal translocation between chromosomes 1 and 14 [46,XX,t(1;14) (q32;q32)]. Both of her parents were phenotypically normal. Molecular studies were carried out to determine the parental origin of chromosomes 1, 13, and 14 in the patient. Using probes for D14S13 and ...

      Known for Chromosome 14 | Uniparental Heterodisomy | Robertsonian Translocation | Human Pair | Phenotypically Abnormal
      KOL-Index: 7764

      Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disease caused by loss of activity of α-l-iduronidase and attendant accumulation of the glycosaminoglycans dermatan sulfate and heparan sulfate. Current treatments are suboptimal and do not address residual disease including corneal clouding, skeletal deformities, valvular heart disease, and cognitive impairment. We treated neonatal dogs with MPS I with intravenous recombinant α-l-iduronidase replacement therapy at the ...

      Known for Mucopolysaccharidosis Type | Enzyme Therapy | Patients Mps | Lysosomal Storage | Antibody Response
      KOL-Index: 7747

      Immune responses can interfere with the effective use of therapeutic proteins to treat genetic deficiencies and have been challenging to manage. To address this problem, we adapted and studied methods of immune tolerance used in canine organ transplantation research to soluble protein therapeutics. A tolerization regimen was developed that prevents a strong antibody response to the enzyme alpha-l-iduronidase during enzyme replacement therapy of a canine model of the lysosomal storage ...

      Known for Immune Tolerance | Enzyme Replacement Therapy | Canine Mucopolysaccharidosis | Therapeutic Proteins | Cyclosporin Azathioprine
      KOL-Index: 5686

      Existing methodologies have been combined to produce a directed approach to the isolation of human genes that escape X inactivation. A mouse-human somatic cell hybrid line was established that has an inactive X as its only human chromosome, and nuclear RNA from this cell line was used to construct a cDNA library. Transcribed human sequences were isolated by screening the library with labeled human DNA. The corresponding genomic sequences were isolated in phage or cosmid clones, and exons ...

      Known for Human Genes | Inactive Chromosome | Situ Hybridization | Dosage Compensation | Hybrid Cells
      KOL-Index: 5664

      An apparently balanced de novo translocation between chromosomes X and 20, 46,X,t(X;20)(Xp20q;Xq20p), was identified in a severely retarded 13-year-old female with macrocephaly, bilateral overfolded pinnae, elbow contractures, clinodactyly, and seizures. BudR-pulse studies show the normal X chromosome to be late replicating in both lymphocytes (50 cells) and skin fibroblasts (25 cells). An HPRT deficient Chinese hamster line was fused with lymphocytes from the patient, and hybrid lines ...

      Known for Human Chromosome | Adenosine Deaminase | 20 Translocation | Inosine Triphosphatase | Somatic Cell
      KOL-Index: 5482

      Central nervous system disease can have devastating consequences in the severe or Hurler form of mucopolysaccharisosis I (MPS I). Intravenously administered recombinant human alpha-L-iduronidase (rhIDU) is not expected to reach and treat the brain disease due to the blood-brain barrier. To determine whether administration of rhIDU into the cerebrospinal fluid could successfully treat the brain, we studied intraventricular administration of rhIDU in rats. RhIDU was stereotactically ...

      Known for Recombinant Human | Rhidu Brain | Lateral Ventricle | Cerebrospinal Fluid | Intraventricular Administration
      KOL-Index: 5382

      Intrathecal enzyme replacement therapy is an experimental option to treat central nervous system disease due to lysosomal storage. Previous work shows that MPS I dogs receiving enzyme replacement with recombinant human alpha-l-iduronidase into the cisterna magna showed normal brain glycosaminoglycan (GAG) storage after three or four doses. We analyzed MPS I dogs that received intrathecal enzyme in a previous study using an assay that detects only pathologic GAG (pGAG). To quantify pGAG ...

      Known for Replacement Therapy | Canine Mucopolysaccharidosis | Intrathecal Enzyme | Mps Dogs | Immune Response
      KOL-Index: 5121

      Satellited marker chromosomes were identified in four individuals from unrelated families; one was first encountered in cultured amniotic fluid cells obtained for prenatal diagnostic studies. We present cytogenetic characterization of these marker chromosomes and clinical findings in the individuals carrying them. Identification of a marker chromosome in amniotic fluid cell cultures presents problems in genetic counseling, as it is often difficult to determine the clinical significance ...

      Known for Marker Chromosomes | Cytogenetic Characterization | Prenatal Diagnosis | Genetic Counseling | Amniotic Fluid
      KOL-Index: 4865

      Sulfate transport was examined in rat liver lysosomes that were isolated from thyroid hormone-treated, thyroidectomized, and control animals. Sulfate uptake was significantly decreased in lysosomes from animals that had received intraperitoneal T3 (3,5,3'-triiodothyronine) at a dose of 20 micrograms/100 g body weight. The effect of T3 was maximal by 24 h post-injection and resulted in marked decreases in both Vmax (control: 155 +/- 33 pmol/unit of beta-hexosaminidase/30 s versus T3 ...

      Known for Thyroid Hormone | Sulfate Transport | Rat Liver Lysosomes | Hexosaminidase 30 | Animals Biological
      KOL-Index: 4610

      Meiotic studies were undertaken in a 24-year-old male patient with short stature, chondrodysplasia punctata, ichthyosis, steroid sulfatase deficiency, and mild mental retardation with an inherited cytologically visible deletion of distal Xp. Molecular investigations showed that the pseudoautosomal region as well as the steroid sulfatase gene were deleted, but telomeric sequences were present at the pter on the deleted X chromosome. A complete failure of sex-chromosome pairing was ...

      Known for Pseudoautosomal Region | Chromosome Pairing | Male Meiosis | Telomeric Sequences | Short Stature

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      Department of Pediatrics, Los Angeles Biomedical Research Institute at Harbor‐UCLA Medical Center, Torrance, CA, USA | Department of Pediatrics, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, CA, United States | De

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