Ian Holt

Ikerbasque — Spain

Biochemistry, Genetics and Molecular Biology · Life Sciences

66h-index14.7kcitations369works2.52yr avg
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Research Topics

Mitochondrial Function and Pathology(124), Metabolism and Genetic Disorders(91), ATP Synthase and ATPases Research(42), RNA Research and Splicing(31), DNA Repair Mechanisms(30)

Publications369 total

Aspartame decreases fat deposits in mice at a cost of mild cardiac hypertrophy and reduced cognitive performance
Biomedicine & Pharmacotherapy·2025Open Access
Characterising PMP22-Proximal Partners in a Schwann Cell Model of Charcot–Marie–Tooth Disease Type1A
Biology·2025Open Access
Dysregulated FOXO1 activity drives skeletal muscle intrinsic dysfunction in amyotrophic lateral sclerosis
Acta Neuropathologica·2024· 5 citedOpen Access
Dysregulated FOXO1 activity drives skeletal muscle intrinsic dysfunction in amyotrophic lateral sclerosis
2024Open Access
Deep learning-driven neuromorphogenesis screenings identify repurposable drugs for mitochondrial disease
2024· 7 citedOpen Access
Proteomic characterization of human LMNA-related congenital muscular dystrophy muscle cells
Neuromuscular Disorders·2024· 2 citedOpen Access
Mitochondrial DNA competition: starving out the mutant genome
Trends in Pharmacological Sciences·2024· 5 citedOpen Access
Energy scarcity and impaired mitochondrial translation induce perinuclear stress granule clustering
2024· 1 citedOpen Access
Lipid Dysregulation Unveil the Intricate Interplay of Lysosomal and Mitochondrial Changes in Frontotemporal Dementia with GRN Haploinsufficiency
2024Open Access
Metabolic Malfunction Mars Muscle Mitochondria
Neurology Genetics·2024Open Access
Elevated cholesterol in <i>ATAD3</i> mutants is a compensatory mechanism that leads to membrane cholesterol aggregation
Brain·2024· 7 citedOpen Access
The Proteome Signatures of Fibroblasts from Patients with Severe, Intermediate and Mild Spinal Muscular Atrophy Show Limited Overlap
Cells·2022· 9 citedOpen Access
2 deoxy-D-glucose augments the mitochondrial respiratory chain in heart
Scientific Reports·2022· 15 citedOpen Access
Defects of Nutrient Signaling and Autophagy in Neurodegeneration
Frontiers in Cell and Developmental Biology·2022· 20 citedOpen Access
Disorders of Replication, Transcription and Translation of Mitochondrial DNA
2022
R-Loops and Mitochondrial DNA Metabolism
Methods in molecular biology·2022· 14 cited
2-Deoxy-D-glucose couples mitochondrial DNA replication with mitochondrial fitness and promotes the selection of wild-type over mutant mitochondrial DNA
Nature Communications·2021· 24 citedOpen Access
CONGENITAL MUSCULAR DYSTROPHIES
Neuromuscular Disorders·2021
OTHER NMDs
Neuromuscular Disorders·2021Open Access
ATAD3 gene-familian de novo duplikazioek Harel-Yoon sindromea sortzen dute, eta kolesterolaren eta mitokondrien metabolismoan kalteak eragiten dituzte
2021
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Frequent Co-authors

Aurelio Reyes(17), Howard T. Jacobs(13), Johannes N. Spelbrink(8), A. E. Harding(7), Takehiro Yasukawa(6), Jiuya He(6), Miriam Di Re(6), Mingyao Yang(5), Hiroshi Sembongi(5), J A Morgan-Hughes(4), Lawrence Kazak(4), Tricia J. Cluett(4), Joanna Poulton(4), Ian M. Fearnley(4), John E. Walker(4), Donald R. Dunbar(4), Jaakko Pohjoismäki(4), Anthony H.V. Schapira(3), Mark Bowmaker(3), Caroline Granycome(3)