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A. Fidziańska · SciRah

Researcher profile · Semantic Scholar

A. Fidziańska

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A. Fidziańska — researcher profile on SciRah

h-index: 23
Works: 126
Citations: 2,332

Most-cited papers

Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome).
M. Tristani-Firouzi, J. L. Jensen, M. R. Donaldson, V. Sansone +9 more
2002Journal of Clinical Investigation555 citations Open access
Semantic Scholar
Ultrastructural abnormality of sarcolemmal nuclei in Emery-Dreifuss muscular dystrophy (EDMD).
A. Fidziańska, D. Toniolo, Irena Hausmanowa-Petrusewicz
1998Journal of Neurological Sciences125 citations
Semantic Scholar
Architectural abnormalities in muscle nuclei. Ultrastructural differences between X-linked and autosomal dominant forms of EDMD.
A. Fidziańska, I. Hausmanowa-Petrusewicz
2003Journal of Neurological Sciences104 citations
Semantic Scholar
Genetic and ultrastructural studies in dilated cardiomyopathy patients: a large deletion in the lamin A/C gene is associated with cardiomyocyte nuclear envelope disruption
P. Gupta, Z. Bilińska, N. Sylvius, Emilie Boudreau +12 more
2010Basic Research in Cardiology98 citations Open access
Semantic Scholar
In vivo and in vitro examination of the functional significances of novel lamin gene mutations in heart failure patients
N. Sylvius, Z. Bilińska, J. Veinot, A. Fidziańska +11 more
2005Journal of Medical Genetics94 citations Open access
Semantic Scholar
Small deletions disturb desmin architecture leading to breakdown of muscle cells and development of skeletal or cardioskeletal myopathy
A. Kaminska, S. Strelkov, B. Goudeau, M. Olivé +9 more
2004Human Genetics60 citations
Semantic Scholar
Myelin composition of spinal cord in a model of amyotrophic lateral sclerosis (ALS) in SOD1G93A transgenic rats.
I. Niebroj-Dobosz, J. Rafałowska, A. Fidziańska, R. Gadamski +1 more
2007Folia Neuropathologica50 citations
Semantic Scholar
Autophagy in transition from hypertrophic cardiomyopathy to heart failure.
A. Fidziańska, Z. Bilińska, E. Walczak, A. Witkowski +1 more
2010Journal of Electron Microscopy49 citations
Semantic Scholar
Molecular genetic and genetic correlations in sodium channelopathies: lack of founder effect and evidence for a second gene.
Jiebiao Wang, J. Zhou, S. Todorovic, W. Feero +14 more
1993American Journal of Human Genetics44 citations
Semantic Scholar
TPM3 mutation in one of the original cases of cap disease
M. Ohlsson, A. Fidziańska, H. Tajsharghi, A. Oldfors
2009Neurology43 citations
Semantic Scholar
Motoneuron death in normal and spinal muscular atrophy-affected human fetuses
A. Fidziańska, J. Rafałowska
2002Acta Neuropathologica43 citations
Semantic Scholar
Cardiovascular magnetic resonance findings in a case of Danon disease
D. Piotrowska-Kownacka, Lukasz Kownacki, M. Kuch, E. Walczak +3 more
2009Journal of Cardiovascular Magnetic Resonance42 citations Open access
Semantic Scholar
A dystrophin missense mutation showing persistence of dystrophin and dystrophin‐associated proteins yet a severe phenotype
Laura R. Goldberg, I. Hausmanowa-Petrusewicz, A. Fidziańska, D. Duggan +2 more
1998Annals of Neurology41 citations
Semantic Scholar
Human ontogenesis. 3. Cell death in fetal muscle.
A. Fidziańska, H. Goebel
1991Acta Neuropathologica38 citations
Semantic Scholar
Main steps of skeletal muscle development in the human: morphological analysis and ultrastructural characteristics of developing human muscle.
N. Romero, M. Mezmezian, A. Fidziańska
2013Handbook of Clinical Neurology37 citations
Semantic Scholar
Coexistence of X-linked recessive Emery-Dreifuss muscular dystrophy with inclusion body myositis-like morphology
A. Fidziańska, K. Rowińska‐Marcińska, Irena Hausmanowa-Petrusewicz
2004Acta Neuropathologica37 citations
Semantic Scholar
Severe phenotypes of SMARD1 associated with novel mutations of the IGHMBP2 gene and nuclear degeneration of muscle and Schwann cells.
M. Jędrzejowska, A. Madej-Pilarczyk, A. Fidziańska, H. Mierzewska +10 more
2014European journal of paediatric neurology30 citations
Semantic Scholar
A novel desmin R355P mutation causes cardiac and skeletal myopathy.
A. Fidziańska, J. Kotowicz, M. Sadowska, B. Goudeau +3 more
2005Neuromuscular Disorders29 citations
Semantic Scholar
"Cap disease"--a failure in the correct muscle fibre formation.
A. Fidziańska
2002Journal of Neurological Sciences29 citations
Semantic Scholar
Obliteration of cardiomyocyte nuclear architecture in a patient with LMNA gene mutation.
A. Fidziańska, Z. Bilińska, F. Tesson, T. Wagner +4 more
2008Journal of Neurological Sciences26 citations
Semantic Scholar
Atypical motor unit potentials in Emery-Dreifuss muscular dystrophy (EDMD).
K. Rowińska-Marcińska, E. Szmidt-Sałkowska, A. Fidziańska, E. Zalewska +3 more
2005Clinical Neurophysiology26 citations
Semantic Scholar
CADASIL or CADVaSIL?
J. Rafałowska, A. Fidziańska, D. Dziewulska, A. Podlecka +2 more
2004Neuropathology (Kyoto. 1993)23 citations
Semantic Scholar
Human ontogenesis
A. Fidziańska, H. Goebel
2004Acta Neuropathologica23 citations
Semantic Scholar
Familial inclusion body myopathy with desmin storage
A. Fidziańska, H. Drac, A. Kaminska
1999Acta Neuropathologica19 citations
Semantic Scholar
Abnormal chaperone-mediated autophagy (CMA) in cardiomyocytes of a boy with Danon disease.
A. Fidziańska, E. Walczak, M. Walski
2007Folia Neuropathologica18 citations
Semantic Scholar
CADASIL: new cases and new questions
J. Rafałowska, A. Fidziańska, D. Dziewulska, A. Podlecka +2 more
2003Acta Neuropathologica18 citations
Semantic Scholar
IBM-type inclusions in a patient with slow-channel syndrome caused by a mutation in the AChR epsilon subunit.
A. Fidziańska, B. Ryniewicz, Xin-Ming Shen, A. Engel
2005Neuromuscular Disorders17 citations
Semantic Scholar
CADASIL: what component of the vessel wall is really a target for Notch 3 gene mutations?
J. Rafałowska, D. Dziewulska, A. Fidziańska
2004Neurological Research17 citations
Semantic Scholar
The Frequency of c.550delA Mutation of the CANP3 Gene in the Polish LGMD2A Population
M. Dorobek, B. Ryniewicz, D. Kabzińska, A. Fidziańska +2 more
2015Genetic Testing and Molecular Biomarkers15 citations
Semantic Scholar
Progeria caused by a rare LMNA mutation p.S143F associated with mild myopathy and atrial fibrillation.
A. Madej-Pilarczyk, T. Kmieć, A. Fidziańska, J. Rękawek +5 more
2008European journal of paediatric neurology15 citations
Semantic Scholar
Suicide muscle cell programme-apoptosis. Ultrastructural study.
A. Fidziańska
2002Folia Neuropathologica15 citations
Semantic Scholar
LMNA mutations in Polish patients with dilated cardiomyopathy: prevalence, clinical characteristics, and in vitro studies
M. Saj, Z. Bilińska, A. Tarnowska, Agnieszka Sioma +14 more
2013BMC Medical Genetics14 citations Open access
Semantic Scholar
Congenital myopathy with abundant ring fibres, rimmed vacuoles and inclusion body myositis-type inclusions.
A. Fidziańska, A. Kaminska
2003Neuropediatrics14 citations
Semantic Scholar
Rimmed vacuoles with beta-amyloid and tau protein deposits in the muscle of children with hereditary myopathy
A. Fidziańska, Z. Glinka
2006Acta Neuropathologica13 citations
Semantic Scholar
Progression of morphological changes within CNS in a transgenic rat model of familial amyotrophic lateral sclerosis.
J. Rafałowska, A. Fidziańska, D. Dziewulska, R. Gadamski +2 more
2006Folia Neuropathologica13 citations
Semantic Scholar
Expression of emerin and lamins in muscle of patients with different forms of Emery-Dreifuss muscular dystrophy.
I. Niebroj-Dobosz, A. Fidziańska, I. Hausmanowa-Petrusewicz
2003Acta myologica13 citations
Semantic Scholar
Limb-girdle muscular dystrophy with severe heart failure overlapping with lipodystrophy in a patient with LMNA mutation p.Ser334del
A. Madej-Pilarczyk, A. Niezgoda, M. Janus, R. Wojnicz +4 more
2016Journal of Applied Genetics12 citations Open access
Semantic Scholar
Nuclear architecture remodelling in envelopathies.
A. Fidziańska, Z. Glinka
2007Folia Neuropathologica12 citations
Semantic Scholar
Correlative biochemical and morphological studies of myelination in human ontogenesis
I. Niebroj-Dobosz, A. Fidziańska, Janina Rafaŀowska, E. Sawicka
2004Acta Neuropathologica12 citations
Semantic Scholar
Ultrastructural evidence of myocardial capillary remodeling in peripartum cardiomyopathy.
A. Fidziańska, E. Walczak, Z. Glinka, G. Religa +2 more
2010Medical Science Monitor11 citations
Semantic Scholar
Early onset Charcot-Marie-Tooth disease caused by a homozygous Leu239Phe mutation in the GDAP1 gene.
Dagmara Kabzińska, H. Drac, K. Rowińska‐Marcińska, A. Fidziańska +2 more
2006Acta myologica11 citations
Semantic Scholar
Correlative biochemical and morphological studies of myelination in human ontogenesis
I. Niebroj-Dobosz, A. Fidziańska, Janina Rafaŀowska, E. Sawicka
2004Acta Neuropathologica11 citations
Semantic Scholar
Novel c.191C>G (p.Pro64Arg) MPV17 mutation identified in two pairs of unrelated Polish siblings with mitochondrial hepatoencephalopathy
Dorota Piekutowska-Abramczuk, M. Pronicki, K. Strawa, A. Karkucińska-Więckowska +10 more
2014Clinical Genetics10 citations
Semantic Scholar
Dilated cardiomyopathy caused by LMNA mutations. Clinical and morphological studies.
Z. Bilińska, N. Sylvius, J. Grzybowski, A. Fidziańska +10 more
2006Kardiologia polska10 citations
Semantic Scholar
Congenital Myopathy with Tubular Aggregates and Tubulofilamentous IBM-Type Inclusions
A. Fidziańska, A. Kaminska, B. Ryniewicz
2005Neuropediatrics10 citations
Semantic Scholar
Ultrastructural Changes in the Skeletal Muscle of Senile Rats with Significant Age-Dependent Motor Deficits
A. Kaminska, A. Fidziańska, G. Schulze, H. Coper +2 more
20029 citations
Semantic Scholar
Spinal muscular atrophy in childhood.
A. Fidziańska
1996Seminars in Pediatric Neurology9 citations
Semantic Scholar
Neonatal Form of Nemaline Myopathy, Muscle Immaturity, and a Microvascular Injury
A. Fidziańska, H. Goebel, M. Kleine
1990Journal of Child Neurology9 citations
Semantic Scholar
Late form of Pompe disease with glycogen storage in peripheral nerves axons.
A. Fidziańska, A. Ługowska, A. Tylki-Szymańska
2011Journal of Neurological Sciences8 citations
Semantic Scholar
Ultrastructural changes in lumbar spinal cord in transgenic SOD1G93A rats.
A. Fidziańska, R. Gadamski, J. Rafałowska, H. Chrzanowska +1 more
2006Folia Neuropathologica8 citations
Semantic Scholar

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D. Dziewulska×4

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Irena Hausmanowa-Petrusewicz×3