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Filippo M. Santorelli

Fondazione Stella Maris ·
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Area of research
Cellular and Molecular Neuroscience · Molecular Biology
Research interest
Research interests include Mitochondrial Function and Pathology, Hereditary Neurological Disorders, Genetic Neurodegenerative Diseases, and Metabolism and Genetic Disorders.
h-index
76
citations
22,488
works
843
NIH funding
primary concept
email

Recent publications

A new variant in the UCHL1 gene supporting its implication in late-onset ataxia with optic atrophy
Neurological Sciences 2025cited by 0position: middledoi
Spinocerebellar ataxia 27B: a frequent and slowly progressive autosomal-dominant cerebellar ataxia—experience from an Italian cohort
Journal of Neurology 2024cited by 21position: lastdoi
GAA-<i>FGF14</i> ataxia (SCA27B): phenotypic profile, natural history progression and 4-aminopyridine treatment response
Brain 2023cited by 110position: middledoi
Responsiveness of the Scale for the Assessment and Rating of Ataxia and Natural History in 884 Recessive and Early Onset Ataxia Patients
Annals of Neurology 2023cited by 35position: middledoi
Extreme phenotypic heterogeneity in non-expansion spinocerebellar ataxias
The American Journal of Human Genetics 2023cited by 31position: middledoi
The clinical and molecular spectrum of the KDM6B-related neurodevelopmental disorder
The American Journal of Human Genetics 2023cited by 30position: middledoi
Autosomal Recessive Cerebellar Ataxias in Europe: Frequency, Onset, and Severity in 677 Patients
Movement Disorders 2023cited by 24position: middledoi
Developmental epileptic encephalopathy in <i>DLG4</i>‐related synaptopathy
Epilepsia 2023cited by 14position: middledoi
PPAR-gamma agonist pioglitazone recovers mitochondrial quality control in fibroblasts from PITRM1-deficient patients
Frontiers in Pharmacology 2023cited by 8position: middledoi
De Novo and Dominantly Inherited <scp><i>SPTAN1</i></scp> Mutations Cause Spastic Paraplegia and Cerebellar Ataxia
Movement Disorders 2022cited by 25position: middledoi
Biallelic variants in <i>HPDL</i> cause pure and complicated hereditary spastic paraplegia
Brain 2021cited by 50position: middledoi
The ARCA Registry: A Collaborative Global Platform for Advancing Trial Readiness in Autosomal Recessive Cerebellar Ataxias
Frontiers in Neurology 2021cited by 33position: middledoi
New pathogenic variants in COQ4 cause ataxia and neurodevelopmental disorder without detectable CoQ10 deficiency in muscle or skin fibroblasts
Journal of Neurology 2021cited by 30position: middledoi
NGS in Hereditary Ataxia: When Rare Becomes Frequent
International Journal of Molecular Sciences 2021cited by 29position: lastdoi
Efficient Neuroprotective Rescue of Sacsin-Related Disease Phenotypes in Zebrafish
International Journal of Molecular Sciences 2021cited by 27position: lastdoi
Movement Disorders in Children with a Mitochondrial Disease: A Cross-Sectional Survey from the Nationwide Italian Collaborative Network of Mitochondrial Diseases
Journal of Clinical Medicine 2021cited by 21position: lastdoi
POLR3A variants in hereditary spastic paraparesis and ataxia: clinical, genetic, and neuroradiological findings in a cohort of Italian patients
Neurological Sciences 2021cited by 21position: lastdoi
High-throughput imaging of ATG9A distribution as a diagnostic functional assay for adaptor protein complex 4-associated hereditary spastic paraplegia
Brain Communications 2021cited by 19position: middledoi
Functional Network Profiles in ARSACS Disclosed by Aptamer-Based Proteomic Technology
Frontiers in Neurology 2021cited by 14position: lastdoi
The Genetic Landscape of Dystrophin Mutations in Italy: A Nationwide Study
Frontiers in Genetics 2020cited by 84position: middledoi
Genotype–phenotype correlations in recessive titinopathies
Genetics in Medicine 2020cited by 79position: middledoi
Impaired flickering of the permeability transition pore causes SPG7 spastic paraplegia
EBioMedicine 2020cited by 55position: middledoi
Clinical, neuropathological, and genetic characterization of STUB1 variants in cerebellar ataxias: a frequent cause of predominant cognitive impairment
Genetics in Medicine 2020cited by 49position: middledoi
Estimating the impact of COVID-19 pandemic on services provided by Italian Neuromuscular Centers: an Italian Association of Myology survey of the acute phase.
PubMed 2020cited by 36position: middledoi
Defining the clinical, molecular and imaging spectrum of adaptor protein complex 4-associated hereditary spastic paraplegia
Brain 2019cited by 75position: middledoi
Adaptor protein complex 4 deficiency: a paradigm of childhood-onset hereditary spastic paraplegia caused by defective protein trafficking
Human Molecular Genetics 2019cited by 73position: middledoi
Molecular Genetics and Interferon Signature in the Italian Aicardi Goutières Syndrome Cohort: Report of 12 New Cases and Literature Review
Journal of Clinical Medicine 2019cited by 43position: middledoi
CLN8 is an endoplasmic reticulum cargo receptor that regulates lysosome biogenesis
Nature Cell Biology 2018cited by 109position: middledoi
Interpreting Genetic Variants in Titin in Patients With Muscle Disorders
JAMA Neurology 2018cited by 93position: middledoi
Next Generation Molecular Diagnosis of Hereditary Spastic Paraplegias: An Italian Cross-Sectional Study
Frontiers in Neurology 2018cited by 83position: lastdoi

Grants

No grants ingested yet.

Frequent collaborators

Enrico Bertini · Royal Brompton Hospital5 papers (2012–2021) · 4 papers (2012–2025) · 4 papers (2013–2018) · 4 papers (2016–2021)Andreas Traschütz · University of Tübingen3 papers (2023–2023)Lüdger Schöls · German Center for Neurodegenerative Diseases3 papers (2023–2023)Maria Marchese · Sunnybrook Health Science Centre3 papers (2016–2021)Stefano Sartori · University of Padua3 papers (2013–2018)Stephan Züchner · Harvard University3 papers (2016–2023) · 3 papers (2013–2018) · 3 papers (2021–2021)Matthis Synofzik · University of Antwerp3 papers (2023–2023)Jonathan Baets · KU Leuven2 papers (2023–2023) · 2 papers (2012–2021)Leonardo Salviati · University of Padua2 papers (2012–2021) · 2 papers (2021–2021)Christoph Kamm · Medizinische Hochschule Hannover2 papers (2023–2023)Mathieu Anheim · Institut de Biologie Moléculaire et Cellulaire2 papers (2023–2023) · 2 papers (2021–2021) · 2 papers (2012–2012)
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