George Karpati

Research.com Recognitions

• 1999 - Fellow of the Royal Society of Canada Academy of Science

Overview

Best Publications

• Inclusion body myositis and myopathies.

  Robert C. Griggs;Valerie Askanas;Salvatore DiMauro;Andrew Engel

  929
  Citations

• The Duchenne muscular dystrophy gene product is localized in sarcolemma of human skeletal muscle

  Elizabeth E. Zubrzycka-Gaarn;Dennis E. Bulman;George Karpati;Arthur H. M. Burghes

  813
  Citations

• The UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase gene is mutated in recessive hereditary inclusion body myopathy

  Iris Eisenberg;Nili Avidan;Tamara Potikha;Hagit Hochner

  602
  Citations

• The syndrome of systemic carnitine deficiency. Clinical, morphologic, biochemical, and pathophysiologic features.

  George Karpati;Stirling Carpenter;Andrew G. Engel;Gordon Watters

  490
  Citations

• Clinical Phenotypes of Different MPZ (P0) Mutations May Include Charcot–Marie–Tooth Type 1B, Dejerine–Sottas, and Congenital Hypomyelination

  Laura E Warner;Max J Hilz;Stanley H Appel;James M Killian

  445
  Citations

• Distribution and threshold expression of the tRNA(Lys) mutation in skeletal muscle of patients with myoclonic epilepsy and ragged-red fibers (MERRF).

  L Boulet;G Karpati;E A Shoubridge

  395
  Citations

• Expression of immunoreactive major histocompatibility complex products in human skeletal muscles

  George Karpati;Yannick Pouliot;Stirling Carpenter

  382
  Citations

• Myoblast transfer in duchenne muscular dystrophy

  George Karpati;Djordje Ajdukovic;Douglas Arnold;Robert B. Gledhill

  378
  Citations

• A distinct form of adult polyglucosan body disease with massive involvement of central and peripheral neuronal processes and astrocytes: a report of four cases and a review of the occurrence of polyglucosan bodies in other conditions such as Lafora's disease and normal ageing.

  Yvon Robitaille;Stirling Carpenter;George Karpati;Salvatore Dimauro

  324
  Citations

• Small‐caliber skeletal muscle fibers do not suffer necrosis in mdx mouse dystrophy

  George Karpati;Stirling Carpenter;Steven Prescott

  311
  Citations

• Dystrophin-deficient mdx muscle fibers are preferentially vulnerable to necrosis induced by experimental lengthening contractions

  Boaz Weller;George Karpati;Stirling Carpenter

  306
  Citations

• Deletion mutants are functionally dominant over wild-type mitochondrial genomes in skeletal muscle fiber segments in mitochondrial disease

  Eric A. Shoubridge;George Karpati;Kenneth E.M. Hastings;Kenneth E.M. Hastings

  303
  Citations

• Mutations in the gene-encoding SERCA1, the fast-twitch skeletal muscle sarcoplasmic reticulum Ca2+ ATPase, are associated with Brody disease.

  Alex Odermatt;Peter E. M. Taschner;Vijay K. Khanna;Herman F. M. Busch

  301
  Citations

• Dystrophin is expressed in mdx skeletal muscle fibers after normal myoblast implantation.

  G. Karpati;Y. Pouliot;E. Zubrzycka-Gaarn;S. Carpenter

  282
  Citations

• Emergence of Early Region 1-Containing Replication-Competent Adenovirus in Stocks of Replication-Defective Adenovirus Recombinants (ΔE1 + ΔE3) During Multiple Passages in 293 Cells

  H. Lochmuller;A. Jani;Johnny Huard;S. Prescott

  282
  Citations

• MYOCLONUS EPILEPSY AND RAGGED-RED FIBRES (MERRF)1. A CLINICAL, PATHOLOGICAL, BIOCHEMICAL, MAGNETIC RESONANCE SPECTROGRAPHIC AND POSITRON EMISSION TOMOGRAPHIC STUDY

  S. F. Berkovic;S. Carpenter;A. Evans;G. Karpati

  276
  Citations

• Duchenne muscular dystrophy: plasma membrane loss initiates muscle cell necrosis unless it is repaired.

  Stirling Carpenter;George Karpati

  270
  Citations

• A Novel Heteroplasmic tRNAleu(CUN) mtDNA Point Mutation in a Sporadic Patient With Mitochondrial Encephalomyopathy Segregates Rapidly in Skeletal Muscle and Suggests an Approach to Therapy

  Katherine Fu;Rebecca Hartlen;Timothy Johns;Angela Genge

  223
  Citations

• Dystrophin-deficient cardiomyocytes are abnormally vulnerable to mechanical stress-induced contractile failure and injury

  Gawiyou Danialou;Alain S. Comtois;Roy Dudley;George Karpati

  222
  Citations

• Short‐term dichloroacetate treatment improves indices of cerebral metabolism in patients with mitochondrial disorders

  N. De Stefano;P. M. Matthews;B. Ford;A. Genge

  219
  Citations