Ferdinando Squitieri

Summary

Affiliation: IRCCS Neuromed
Country: Italy

Publications

  1. ncbi Distinct brain volume changes correlating with clinical stage, disease progression rate, mutation size, and age at onset prediction as early biomarkers of brain atrophy in Huntington's disease
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed and Centre for Rare Diseases, Localita Camerelle, Pozzilli Is, Italy
    CNS Neurosci Ther 15:1-11. 2009
  2. ncbi Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins
    Ferdinando Squitieri
    Neurogenetics Unit and Centre for Rare Disease, IRCCS Neuromed, Localita Camerelle, 86077, Pozzilli, Italy
    Eur J Nucl Med Mol Imaging 36:1113-20. 2009
  3. ncbi One-year safety and tolerability profile of pridopidine in patients with Huntington disease
    Ferdinando Squitieri
    Centre for Neurogenetics and Rare Diseases, IRCCS Neuromed, Pozzilli, Italy
    Neurology 80:1086-94. 2013
  4. ncbi Huntington's disease: how intermediate are intermediate repeat lengths?
    Ferdinando Squitieri
    Neurogenetics and Rare Diseases Centre, IRCCS Neuromed, Pozzilli, Italy
    Mov Disord 27:1714-7. 2012
  5. ncbi Abnormal morphology of peripheral cell tissues from patients with Huntington disease
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed and Centre for Rare Diseases, Localita Camerelle, 86077, Pozzilli Is, Italy
    J Neural Transm 117:77-83. 2010
  6. ncbi Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation
    Vittorio Maglione
    Neurogenetics Unit, IRCCS INM Neuromed, , Pozzilli, IS, Italy
    Mech Ageing Dev 127:213-6. 2006
  7. ncbi Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed, Pozzilli, IS, Italy
    Mech Ageing Dev 127:217-20. 2006
  8. ncbi DNA instability in replicating Huntington's disease lymphoblasts
    Milena Cannella
    Neurogenetics Unit, IRCCS Neuromed, Pozzilli, IS, Italy
    BMC Med Genet 10:11. 2009
  9. ncbi Genotype-, aging-dependent abnormal caspase activity in Huntington disease blood cells
    Ferdinando Squitieri
    Neurogenetics Unit and Rare Diseases Centre, IRCCS Neuromed, Pozzilli Is, Italy
    J Neural Transm 118:1599-607. 2011
  10. ncbi Early defect of transforming growth factor β1 formation in Huntington's disease
    Giuseppe Battaglia
    Neuropharmacology Unit, IRCCS Neuromed, Pozzilli, Italy
    J Cell Mol Med 15:555-71. 2011

Collaborators

Detail Information

Publications16

  1. ncbi Distinct brain volume changes correlating with clinical stage, disease progression rate, mutation size, and age at onset prediction as early biomarkers of brain atrophy in Huntington's disease
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed and Centre for Rare Diseases, Localita Camerelle, Pozzilli Is, Italy
    CNS Neurosci Ther 15:1-11. 2009
    ..The progressive CSF increase depended on CAG mutation size and continued linearly until the last stages of HD, perhaps representing the best marker of progression rate and severity in HD (R(2)= 0.25, P < 0.0001)...
  2. ncbi Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins
    Ferdinando Squitieri
    Neurogenetics Unit and Centre for Rare Disease, IRCCS Neuromed, Localita Camerelle, 86077, Pozzilli, Italy
    Eur J Nucl Med Mol Imaging 36:1113-20. 2009
    ....
  3. ncbi One-year safety and tolerability profile of pridopidine in patients with Huntington disease
    Ferdinando Squitieri
    Centre for Neurogenetics and Rare Diseases, IRCCS Neuromed, Pozzilli, Italy
    Neurology 80:1086-94. 2013
    ..To assess the 1-year safety profile of the dopaminergic stabilizer pridopidine in patients with Huntington disease...
  4. ncbi Huntington's disease: how intermediate are intermediate repeat lengths?
    Ferdinando Squitieri
    Neurogenetics and Rare Diseases Centre, IRCCS Neuromed, Pozzilli, Italy
    Mov Disord 27:1714-7. 2012
    ..The expansion mutation in HTT is dominantly transmitted and codes for a protein named huntingtin (htt)...
  5. ncbi Abnormal morphology of peripheral cell tissues from patients with Huntington disease
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed and Centre for Rare Diseases, Localita Camerelle, 86077, Pozzilli Is, Italy
    J Neural Transm 117:77-83. 2010
    ..Moreover, the occurrence of ultrastructural cell pathology reminiscent of neuronal degeneration in HD, suggests the use of human peripheral cells as a tool to investigate the pathogenic cascade subsequent to huntingtin dysregulation...
  6. ncbi Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation
    Vittorio Maglione
    Neurogenetics Unit, IRCCS INM Neuromed, , Pozzilli, IS, Italy
    Mech Ageing Dev 127:213-6. 2006
    ..This data offers a biological explanation to the clinical in-patients evidence of mutation homozygosity associated with more severe phenotype...
  7. ncbi Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed, Pozzilli, IS, Italy
    Mech Ageing Dev 127:217-20. 2006
    ..We argue that early mitochondrial impairment at basal level may affect the severity of HD progression in patients...
  8. ncbi DNA instability in replicating Huntington's disease lymphoblasts
    Milena Cannella
    Neurogenetics Unit, IRCCS Neuromed, Pozzilli, IS, Italy
    BMC Med Genet 10:11. 2009
    ..To test this hypothesis we used, as a model, cultured HD patients' lymphoblasts with various CAG repeat lengths...
  9. ncbi Genotype-, aging-dependent abnormal caspase activity in Huntington disease blood cells
    Ferdinando Squitieri
    Neurogenetics Unit and Rare Diseases Centre, IRCCS Neuromed, Pozzilli Is, Italy
    J Neural Transm 118:1599-607. 2011
    ..Peripheral tissues (i.e. blood cells) may theoretically represent an important tool for studying HD mechanisms and searching for new biomarkers, according to the patients' genotype...
  10. ncbi Early defect of transforming growth factor β1 formation in Huntington's disease
    Giuseppe Battaglia
    Neuropharmacology Unit, IRCCS Neuromed, Pozzilli, Italy
    J Cell Mol Med 15:555-71. 2011
    ....
  11. ncbi The platelet maximum number of A2A-receptor binding sites (Bmax) linearly correlates with age at onset and CAG repeat expansion in Huntington's disease patients with predominant chorea
    Vittorio Maglione
    Neurogenetics Unit, IRCCS INM Neuromed, , 86077 Pozzilli, IS, Italy
    Neurosci Lett 393:27-30. 2006
    ..Further studies on a larger sample size should confirm whether the analysis of A(2A)-receptor binding in patients' blood could be a useful clinical marker according to the patients' phenotype...
  12. ncbi Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS INM Neuromed, Pozzilli Is, Italy
    Brain 126:946-55. 2003
    ..These data, once confirmed in a larger series of patients, point to the possibility that the mechanisms underlying age at onset and disease progression in Huntington disease may differ...
  13. ncbi Juvenile Huntington's disease: does a dosage-effect pathogenic mechanism differ from the classical adult disease?
    Ferdinando Squitieri
    Neurogenetics Unit, IRCCS Neuromed, Pozzilli, IS, Italy
    Mech Ageing Dev 127:208-12. 2006
    ..In this review we discuss the possibility that some of the pathogenic mechanisms contributing to age at onset and progression may differ in the early onset HD compared with the classical adult pathology...
  14. ncbi Validation of the first quality-of-life measurement for patients with Huntington's disease: the Huntington Quality of Life Instrument
    Emilie Clay
    IRCCS Neuromed, Neurogenetics and Rare Diseases Centre, Pozzilli, Italy
    Int Clin Psychopharmacol 27:208-14. 2012
    ..No differential item functioning was detected. External validity supported the scale's robustness. These data support the validity of the H-QoL-I in patients with HD...
  15. ncbi Reduced activity of cortico-striatal fibres in the R6/2 mouse model of Huntington's disease
    Anna Traficante
    Department of Neuroscience, Istituto Neurologico Mediterraneo Neuromed, Localita Camerelle, Pozzilli, Italy
    Neuroreport 18:1997-2000. 2007
    ..These data support the hypothesis of a cortico-striatal dysfunction in Huntington's disease...
  16. ncbi Current Pharmacological Management in Juvenile Huntington's Disease
    Lisa Robertson
    Department of Clinical Genetics, Sheffield Children s Hospital, Sheffield UK S10 2TH Neurogenetics and Rare Diseases Centre, IRCCS Neuromed, Pozzilli Is, Italy University of Cambridge Department of Clinical Neurophysiology, Institute of Psychiatry and Naurology, Warsaw, Poland and Dept of Neurology, University of Ulm, Ulm, Germany
    PLoS Curr 4:RRN1304. 2012
    ..5 patients were taking more than 8 medications.Conclusions: The most commonly prescribed group of medication was the anti-psychotic. Many patients were on multiple therapies, highlighting the need to rationalise medications...