S-M Pulst

Summary

Affiliation: Cedars-Sinai Medical Center
Country: USA

Publications

  1. ncbi request reprint Genetic linkage analysis
    S M Pulst
    Division of Neurology, Cedars Sinai Medical Center, University of California at Los Angeles School of Medicine, 90048, USA
    Arch Neurol 56:667-72. 1999
  2. ncbi request reprint Spinocerebellar ataxia type 2: polyQ repeat variation in the CACNA1A calcium channel modifies age of onset
    Stefan M Pulst
    Division of Neurology, Department of Medicine and Rose Moss Laboratory for Parkinson and Related Diseases, Burns and Allen Research Institute, Cedars Sinai Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA 90048, USA
    Brain 128:2297-303. 2005
  3. ncbi request reprint Identification and expression of the gene for human ataxin-2-related protein on chromosome 16
    Karla P Figueroa
    Rose Moss Neurogenetics Laboratory for Parkinson and Related Diseases, CSMS Burns and Allen Research Institute, Division of Neurology, Cedars Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA
    Exp Neurol 184:669-78. 2003
  4. pmc Neurogenetics: single gene disorders
    S M Pulst
    Division of Neurology, Cedars Sinai Medical Center, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
    J Neurol Neurosurg Psychiatry 74:1608-14. 2003
  5. ncbi request reprint Association of moderate polyglutamine tract expansions in the slow calcium-activated potassium channel type 3 with ataxia
    K P Figueroa
    Division of Neurology, Cedars Sinai Medical Center, Los Angeles, CA 90048, USA
    Arch Neurol 58:1649-53. 2001
  6. ncbi request reprint HRS inhibits EGF receptor signaling in the RT4 rat schwannoma cell line
    Daniel R Scoles
    Rose Moss Neurogenetics Laboratory and Division of Neurology, Burns and Allen Research Institute, Cedars Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA
    Biochem Biophys Res Commun 335:385-92. 2005
  7. ncbi request reprint Neurofibromatosis 2 (NF2) tumor suppressor schwannomin and its interacting protein HRS regulate STAT signaling
    Daniel R Scoles
    Cedars Sinai Medical Center, School of Medicine, University of California at Los Angeles, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA
    Hum Mol Genet 11:3179-89. 2002
  8. ncbi request reprint Effects of Nf2 missense mutations on schwannomin interactions
    Daniel R Scoles
    Division of Neurology, Cedars Sinai Medical Center, 1145E Medical Tower, 8631 West 3rd Street, Los Angeles, CA 90048, USA
    Biochem Biophys Res Commun 290:366-74. 2002
  9. pmc Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat
    Hiushan Ng
    Rose Moss Laboratory for Parkinson and Neurodegenerative Diseases, Burns and Allen Research Institute, and Division of Neurology, Cedars Sinai Medical Center, USA
    Exp Neurol 208:207-15. 2007
  10. ncbi request reprint Generation and characterization of Sca2 (ataxin-2) knockout mice
    Tim Rasmus Kiehl
    Rose Moss Laboratory for Parkinson and Neurodegenerative Diseases, Burns and Allen Research Institute, Cedars Sinai Medical Center, Los Angeles, CA 90048, USA
    Biochem Biophys Res Commun 339:17-24. 2006

Research Grants

  1. Parkin Interacting Proteins
    Stefan Pulst; Fiscal Year: 2006

Collaborators

Detail Information

Publications15

  1. ncbi request reprint Genetic linkage analysis
    S M Pulst
    Division of Neurology, Cedars Sinai Medical Center, University of California at Los Angeles School of Medicine, 90048, USA
    Arch Neurol 56:667-72. 1999
    ....
  2. ncbi request reprint Spinocerebellar ataxia type 2: polyQ repeat variation in the CACNA1A calcium channel modifies age of onset
    Stefan M Pulst
    Division of Neurology, Department of Medicine and Rose Moss Laboratory for Parkinson and Related Diseases, Burns and Allen Research Institute, Cedars Sinai Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA 90048, USA
    Brain 128:2297-303. 2005
    ..Future studies are needed to determine whether the modifier effect of CACNA1A relates to neuronal dysfunction or cell death of Purkinje neurons...
  3. ncbi request reprint Identification and expression of the gene for human ataxin-2-related protein on chromosome 16
    Karla P Figueroa
    Rose Moss Neurogenetics Laboratory for Parkinson and Related Diseases, CSMS Burns and Allen Research Institute, Division of Neurology, Cedars Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA
    Exp Neurol 184:669-78. 2003
    ..A2RP has several isoforms with different C-terminal domains. The longest isoform is composed of 1051 amino acids and has widespread expression in human tissues by Northern and Western blot analyses...
  4. pmc Neurogenetics: single gene disorders
    S M Pulst
    Division of Neurology, Cedars Sinai Medical Center, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
    J Neurol Neurosurg Psychiatry 74:1608-14. 2003
    ..This review covers some of the molecular tools and animal models used for genetic analysis and for DNA based diagnosis, and a brief survey of information available on the internet...
  5. ncbi request reprint Association of moderate polyglutamine tract expansions in the slow calcium-activated potassium channel type 3 with ataxia
    K P Figueroa
    Division of Neurology, Cedars Sinai Medical Center, Los Angeles, CA 90048, USA
    Arch Neurol 58:1649-53. 2001
    ....
  6. ncbi request reprint HRS inhibits EGF receptor signaling in the RT4 rat schwannoma cell line
    Daniel R Scoles
    Rose Moss Neurogenetics Laboratory and Division of Neurology, Burns and Allen Research Institute, Cedars Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA
    Biochem Biophys Res Commun 335:385-92. 2005
    ..This study is the first to show that HRS can reduce the abundance of total and active EGFR and may reflect cell type-specific HRS function...
  7. ncbi request reprint Neurofibromatosis 2 (NF2) tumor suppressor schwannomin and its interacting protein HRS regulate STAT signaling
    Daniel R Scoles
    Cedars Sinai Medical Center, School of Medicine, University of California at Los Angeles, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA
    Hum Mol Genet 11:3179-89. 2002
    ..These data are consistent with the hypothesis that schwannomin requires HRS interaction to be fully functionally active and to inhibit STAT activation...
  8. ncbi request reprint Effects of Nf2 missense mutations on schwannomin interactions
    Daniel R Scoles
    Division of Neurology, Cedars Sinai Medical Center, 1145E Medical Tower, 8631 West 3rd Street, Los Angeles, CA 90048, USA
    Biochem Biophys Res Commun 290:366-74. 2002
    ..We show that unique inter- and intramolecular interactions occur for schwannomin isoform 2, suggesting that this schwannomin isoform has unique functional properties compared to schwannomin isoform 1...
  9. pmc Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat
    Hiushan Ng
    Rose Moss Laboratory for Parkinson and Neurodegenerative Diseases, Burns and Allen Research Institute, and Division of Neurology, Cedars Sinai Medical Center, USA
    Exp Neurol 208:207-15. 2007
    ..These findings suggest that the C-terminal domains are important for ataxin-2 cytotoxicity and that Golgi abnormalities may not be primary in the pathogenic process...
  10. ncbi request reprint Generation and characterization of Sca2 (ataxin-2) knockout mice
    Tim Rasmus Kiehl
    Rose Moss Laboratory for Parkinson and Neurodegenerative Diseases, Burns and Allen Research Institute, Cedars Sinai Medical Center, Los Angeles, CA 90048, USA
    Biochem Biophys Res Commun 339:17-24. 2006
    ..Our results demonstrate that ataxin-2, although widely expressed, is not essential in development or during adult survival in the mouse, but leads to adult-onset obesity...
  11. ncbi request reprint Late-onset Friedreich ataxia: phenotypic analysis, magnetic resonance imaging findings, and review of the literature
    Roongroj Bhidayasiri
    Department of Neurology, UCLA Medical Center, Los Angeles, Calif, USA
    Arch Neurol 62:1865-9. 2005
    ..Late-onset FA (LOFA) is defined as onset after age 25 years...
  12. pmc Parkin is an E3 ubiquitin-ligase for normal and mutant ataxin-2 and prevents ataxin-2-induced cell death
    Duong P Huynh
    Rose Moss Laboratory for Parkinson and Neurodegenerative Diseases, Burns and Allen Research Institute, and Division of Neurology, Cedars Sinai Medical Center, USA
    Exp Neurol 203:531-41. 2007
    ..The role of parkin variants in modifying the SCA2 phenotype and its use as a therapeutic target should be further investigated...
  13. ncbi request reprint Genetic heterogeneity of stably transfected cell lines revealed by expression profiling with oligonucleotide microarrays
    Min Kyu Oh
    Rose Moss Neurogenetics Laboratory for Parkinson and Related Diseases, CSMS Burns and Allen Research Institute, Division of Neurology, Cedars Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California 90048, USA
    J Cell Biochem 90:1068-78. 2003
    ..Based on these analyses, we recommend that replications of experiments with several selected cell lines are necessary to assess biological effects of induced gene expression...
  14. ncbi request reprint Mitochondrial complex I gene variant associated with early age at onset in spinocerebellar ataxia type 2
    David K Simon
    Department of Neurology, Beth Israel Deaconess Medical Center, 77 Avenue Louis Pasteur, Room HIM 847, Boston, MA 02115, USA
    Arch Neurol 64:1042-4. 2007
    ..We hypothesized that this variant might have a protective effect on the central nervous system and therefore might delay the onset of symptoms in spinocerebellar ataxia type 2 (SCA2)...
  15. ncbi request reprint Dystonia (DYT) genetic loci
    Roongroj Bhidayasiri
    Department of Neurology, Reed Neurological Research Institute, UCLA Medical Center, Geffen School of Medicine at UCLA, 710 Westwood Plaza, Los Angeles, CA 90095, USA
    Eur J Paediatr Neurol 9:367-70. 2005

Research Grants1

  1. Parkin Interacting Proteins
    Stefan Pulst; Fiscal Year: 2006
    ..The ultimate goal of this R21 proposal is to further characterize proteins involved in parkin function and to screen these proteins for mutations or susceptibility alleles in PD patients. ..