Kuchuan Chen

A human iPSC-derived motor neuron-myogenic cell coculture platform to evaluate neuromuscular junction innervation after axon injury and in Spinal Muscular Atrophy

Keunjung Heo, Xiangsunze Zeng, Kathleen Zhang, Kuchuan Chen et autres

SUMMARY Traumatic nerve injury is challenging as motor neurons with damaged axons repair slowly, which can lead to muscle degeneration, while in Spinal Muscular Atrophy (SMA), muscle innervation is reduced. While pro-regenerative or neuroprotective compounds have been identified, their specific ability to …

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Non-muscle myosin II inhibition at the site of axon injury increases axon regeneration

Keunjung Heo, Tammy Szu‐Yu Ho, Xiangsunze Zeng, Bruna Lenfers Turnes et autres

Motor axon regeneration following peripheral nerve injury is critical for motor recovery but therapeutic interventions enhancing this are not available. We conduct a phenotypic screen on human motor neurons and identified blebbistatin, a non-muscle myosin II inhibitor, as the most effective neurite …

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Non-muscle Myosin Ii Inhibition at the Site of Axon Injury Increases Axon Regeneration

Clifford J. Woolf, Keunjung Heo, Tammy Szu‐Yu Ho, Xiangsunze Zeng et autres

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Phospholipase PLA2G6, a Parkinsonism-Associated Gene, Affects Vps26 and Vps35, Retromer Function, and Ceramide Levels, Similar to α-Synuclein Gain

Guang Lin, Pei-Tseng Lee, Kuchuan Chen, Dongxue Mao et autres

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Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals

Kuchuan Chen, Tammy Szu‐Yu Ho, Guang Lin, Kai Li Tan et autres

Friedreich’s ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by mutations in Frataxin (FXN). Loss of FXN causes impaired mitochondrial function and iron homeostasis. An elevated production of reactive oxygen species (ROS) was previously proposed to contribute to the pathogenesis of …

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Author response: Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals

Kuchuan Chen, Tammy Szu‐Yu Ho, Guang Lin, Kai Li Tan et autres

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Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

Kuchuan Chen, Guang Lin, Nele A.T Haelterman, Tammy Szu‐Yu Ho et autres

Mutations in Frataxin (FXN) cause Friedreich's ataxia (FRDA), a recessive neurodegenerative disorder. Previous studies have proposed that loss of FXN causes mitochondrial dysfunction, which triggers elevated reactive oxygen species (ROS) and leads to the demise of neurons. Here we describe a ROS …

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Author response: Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

Kuchuan Chen, Guang Lin, Nele A.T Haelterman, Tammy Szu‐Yu Ho et autres

Friedreich’s ataxia is a disorder in which nerve cells in the spinal cord, cerebellum and dorsal root ganglia progressively die as a person ages. People with this disorder often have difficulties with walking and can eventually develop other problems such as heart …

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Ubr3, a Novel Modulator of Hh Signaling Affects the Degradation of Costal-2 and Kif7 through Poly-ubiquitination

Tongchao Li, Junkai Fan, Bernardo Blanco‐Sánchez, Nikolaos Giagtzoglou et autres

Hedgehog (Hh) signaling regulates multiple aspects of metazoan development and tissue homeostasis, and is constitutively active in numerous cancers. We identified Ubr3, an E3 ubiquitin ligase, as a novel, positive regulator of Hh signaling in Drosophila and vertebrates. Hh signaling regulates the …

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Drosophilatools and assays for the study of human diseases

Berrak Uğur, Kuchuan Chen, Hugo Jozef Bellen

Many of the internal organ systems of Drosophila melanogaster are functionally analogous to those in vertebrates, including humans. Although humans and flies differ greatly in terms of their gross morphological and cellular features, many of the molecular mechanisms that govern development and …

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A library of MiMICs allows tagging of genes and reversible, spatial and temporal knockdown of proteins in Drosophila

Sonal Nagarkar-Jaiswal, Pei-Tseng Lee, Megan E. Campbell, Kuchuan Chen et autres

Here, we document a collection of ∼7434 MiMIC (Minos Mediated Integration Cassette) insertions of which 2854 are inserted in coding introns. They allowed us to create a library of 400 GFP-tagged genes. We show that 72% of internally tagged proteins are functional, …

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Author response: A library of MiMICs allows tagging of genes and reversible, spatial and temporal knockdown of proteins in Drosophila

Sonal Nagarkar-Jaiswal, Pei-Tseng Lee, Megan E. Campbell, Kuchuan Chen et autres

A large collection of functional EGFP tagged proteins derived from MiMIC insertions allows examination of protein expression in unfixed tissues and efficient tissue specific reversible knock down of proteins.

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