Magrané Laboratory

Of the Feil Family  Brain and Mind Research Institute
at ​​Weill Cornell Medical College​​


Friedreich’s Ataxia
Our current line of research focuses on Friedreich’s ataxia, a rare genetic mitochondrial disorder. Two research papers from this topic are under preparation for submission. Coming up soon!
- Bolea I, Gan WB, Manfredi G, Magrané J (2014) Imaging of mitochondrial dynamics in motor and sensory axons of living mice. Methods Enzymol, 547:97-110.
Live imaging of mitochondrial axonal transport
A few years ago, we took a novel approach to the study of neuronal and mitochondrial dysfunction in neurodegenerative diseases by using microscopy-based, live imaging assays both in vitro (isolated neurons) and in vivo (whole mouse). Most of the studies were performed in the context of Amyotrophic Lateral Sclerosis (ALS) and in other neurodegenerative diseases in collaboration (
- Magrané J, Cortez C, Gan WB, Manfredi G (2014) Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models. Hum Molec Genet, 23(6):1413-24
Magrané J, Sahawneh MA, Przedborski S, Estevez AG, Manfredi G. (2012) Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. J Neurosci, 32:229-42.
Kim HJ, Magrané J (2011) Isolation and culture of neurons and astrocytes from the mouse brain cortex. Methods Mol Biol. (vol. Neurodegeneration), 793:63-75.
Live imaging of organelle transport in neurons
- Gao J, Naughton SX, Wulff H, Singh V, Beck WD, Magrané J, Thomas B,Kaidery NA, Hernandez CM, and Terry AV Jr. (2015) Diisopropylfluorophosphate impairs the transport of membrane bound organelles in rat cortical axons. J Pharmacol Exp Ther (accepted)
Suárez-Calvet M, Belbin O, Pera M, Badiola N, Magrané J, Guardia-Laguarta C, Muñoz L, Colom M, Clarimón J, Lleó A (2014) Autosomal-dominant Alzheimer’s disease mutations at the same codon of Amyloid Precursor Protein differentially alter Aβ production. J Neurochem, 128(2):330-9.
Guardia-Laguarta C, Area-Gomez E, Rub C, Li Y, Magrané J, Becker D,Voos W, Schon EA, Przedborski S (2014) alpha-Synuclein is localized to mitochondria-associated ER membranes. J Neurosci, 34:249-59.
Kim HJ, Magrané J, Starkov AA, Manfredi G. (2012) The mitochondrial calcium regulator cyclophilin D is an essential component o festrogen mediated neuroprotection in amyotrophic lateral sclerosis. Brain, 135:2865-74.
Fischer LR, Igoudjil A, Magrané J, Li Y, Hansen JM, Manfredi G, Glass JD (2011) SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the SOD1 knockout mouse. Brain 134 (Pt 1):196-209.
Wilk S, Scheibenbogen C, Bauer S,Jenke A, Rother M, Guerreiro M, Kudernatsch R1, Goerner N, Poller W, X Elligsen-Merkel D, Utku N, Magrané J, Volk HD, Skurk C (2011) Adiponectin is a negative regulator of antigen-activated T cells. Eur J Immunol, 41(8):2323-32.
Kawamata H, Tiranti V, Magrané J, Chinopoulos C, Manfredi G (2011) adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria. Hum Molec Genet. 20(15):2964-74.
Fonte V, Dostal V, Roberts CM, Gonzales P, Lacor P, Magrané J, Dingwell N, Fan NY, Silverman MA, Stein G, Link CD (2011) A glycine zipper motif mediates the formation of toxic beta-amyloid oligomers in vitro and in vivo. Mol Neurodegener Aug 23;6(1):61.
Igoudjil*A, Magrané* J, Fischer LR, Kim HJ, Hervias I, Dumont M, Cortez C, Glass JD, Starkov AA, Manfredi G (2011) In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space. J Neurosci, 31:15826-37. *equal contribution