ARNULF HANS WERNER KOEPPEN
- Friedreich's ataxia: pathology, pathogenesis, and molecular geneticsArnulf H Koeppen
VA Medical Center, 113 Holland Ave, and Albany Medical College, 47 New Scotland Ave, Albany, NY 12208, USA
J Neurol Sci 303:1-12. 2011....
- Nikolaus Friedreich and degenerative atrophy of the dorsal columns of the spinal cordArnulf H Koeppen
Neurology and Research Services, VA Medical Center, Departments of Neurology and Pathology, Albany Medical College, Albany, New York 12208, USA
J Neurochem 126:4-10. 2013..It is remarkable that Friedreich also described the abnormal hearts in the disease that now bears his name since hypertrophic cardiomyopathy is now recognized as the main cause of death in Friedreich's ataxia...
- The reciprocal cerebellar circuitry in human hereditary ataxiaArnulf H Koeppen
Research Service, Veterans Affairs Medical Center, 113 Holland Ave, Albany, NY 12208, USA
Cerebellum 12:493-503. 2013..These observations offer a semiquantitative assessment of the critical role of the DN in the ataxic phenotype of SCA and FRDA while other parts of the circuitry appear less important...
- Friedreich ataxia: neuropathology revisedArnulf H Koeppen
Research Service, Veterans Affairs Medical Center, Albany, New York 12208, USA
J Neuropathol Exp Neurol 72:78-90. 2013..In light of the selective vulnerability of organs and tissues to systemic frataxin deficiency, many questions about the pathogenesis of Friedreich ataxia remain...
- Friedreich's ataxia causes redistribution of iron, copper, and zinc in the dentate nucleusArnulf H Koeppen
Research Service 151, Veterans Affairs Medical Center, 113 Holland Ave, Albany, NY, 12208, USA
Cerebellum 11:845-60. 2012..The results suggest that MT can provide effective protection against endogenous Cu and Zn toxicity in FRDA, similar to the neuroprotective sequestration of Fe in holoferritin...
- The dorsal root ganglion in Friedreich's ataxiaArnulf H Koeppen
Research Service 151, Veterans Affairs Medical Center, 113 Holland Ave, Albany, NY 12208, USA
Acta Neuropathol 118:763-76. 2009..The observations support the conclusions that satellite cells and DRG neurons are affected by iron dysmetabolism; and that regeneration and inappropriate myelination of small axons in DR are characteristic of the disease...
- The pathology of superficial siderosis of the central nervous systemArnulf H Koeppen
Research Service, VA Medical Center, Albany, NY 12208, USA
Acta Neuropathol 116:371-82. 2008..The conversion of heme iron to hemosiderin probably involves both translational and transcriptional activation of ferritin biosynthesis...
- The pathogenesis of spinocerebellar ataxiaArnulf H Koeppen
Neurology and Pathology Services, VA Medical Center, Albany, New York 12208, USA
Cerebellum 4:62-73. 2005..The role of polyglutamine-containing intranuclear and cytoplasmic inclusion bodies in SCA remains unknown but protein aggregation may be the common step in the pathogenesis of these otherwise rather heterogeneous disorders...
- A brief history of Pelizaeus-Merzbacher disease and proteolipid proteinArnulf H Koeppen
Neurology Service 127, VA Medical Center, 113 Holland Avenue, Albany, NY 12208, USA
J Neurol Sci 228:198-200. 2005
- Heme oxygenase in experimental intracerebral hemorrhage: the benefit of tin-mesoporphyrinArnulf H Koeppen
Neurology Service, VA Medical Center, Albany, New York 12208, USA
J Neuropathol Exp Neurol 63:587-97. 2004..The ICH disrupted the blood-brain barrier sufficiently to allow SnMP to enter the brain in pharmacological amounts, and the metalloporphyrin provided significant protection against neuronal loss...
- Wallerian degeneration: history and clinical significanceArnulf H Koeppen
Departments of Neurology and Pathology, Albany Medical College, Neurology Service 127, VA Medical Center, Albany, NY 12208, USA
J Neurol Sci 220:115-7. 2004
- A brief history of brain iron researchArnulf H Koeppen
Department of Neurology, Stratton VA Medical Center, 113 Holland Avenue, Albany, NY 12208, USA
J Neurol Sci 207:95-7. 2003
- The neuropathology of late-onset Friedreich's ataxiaArnulf H Koeppen
Cerebellum 10:96-103. 2011..Frataxin levels in the dentate nucleus of two patients with late onset were similar to those of seven patients with juvenile onset...
- The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteinsArnulf H Koeppen
Research, Veterans Affairs Medical Center, Albany, NY 12208, USA
Acta Neuropathol 114:163-73. 2007..Neuronal loss in the dentate nucleus is the likely result of trans-synaptic degeneration...
- Tin-protoporphyrin prevents experimental superficial siderosis in rabbitsArnulf H Koeppen
Neurology Service, VA Medical Center, Albany, New York 12208, USA
J Neuropathol Exp Neurol 61:689-701. 2002..It did not alter the microglial response. The observations allowed the conclusion that SnPP suppressed heme oxidation but did not affect other steps in the pathogenesis of superficial siderosis...
- Pathology and pathogenesis of sensory neuropathy in Friedreich's ataxiaJennifer A Morral
VA Medical Center, Albany, NY 12208, USA
Acta Neuropathol 120:97-108. 2010..Sural nerves in FRDA showed no convincing change in ferritin and ferroportin, militating against local iron dysmetabolism. The result stands out in contrast to the previously reported changes in dorsal spinal roots of patients with FRDA...
- Pelizaeus-Merzbacher diseaseArnulf H Koeppen
Neurology Research Service, Stratton VA Medical Center and Albany Medical College, NY 12208, USA
J Neuropathol Exp Neurol 61:747-59. 2002..Several naturally occurring and transgenic animal models with PLP gene mutations or deletions have contributed to our understanding of dysmyelination in PMD and the general knowledge of myelination and myelin repair...
- Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxiaSusan Michael
Research Service, Veterans Affairs Medical Center, Albany, New York, USA
Cerebellum 5:257-67. 2006..Progressive cardiomyopathy in FRDA is the likely result of iron-catalyzed mitochondrial damage followed by muscle fiber necrosis and a chronic reactive myocarditis...
- Clinical features and neuropathology of autosomal dominant spinocerebellar ataxia (SCA17)Arndt Rolfs
Department of Neurology, University of Rostock, Rostock, Germany
Ann Neurol 54:367-75. 2003..Based on clinical and genetic data, we conclude that SCA17 is rare among white SCA patients. SCA17 should be considered in sporadic and familial cases of ataxia with accompanying psychiatric symptoms and dementia...
- Ocular apraxia in recessive ataxiaArnulf H Koeppen
Arch Neurol 59:874; author reply 874. 2002
- Iron in the pathogenesis of Friedreich's ataxiaARNULF HANS WERNER KOEPPEN; Fiscal Year: 2010..Chelation therapy in an effort to remove iron from affected tissues remains controversial. The proposed investigation will help clarify the potential benefit of chelation therapy in FRDA. ..