Pilaz Lab
@ljpilaz
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Dad, Husband, Scientist, Woodworker, Heavy metal fan. Opinions are my own. @neurowoodworks is my alter ego.
Sioux Falls, SD
Joined September 2014
Beautiful view of the mouse cerebellum, highlighted by Hoechst🔵, RTN4🟢, RTN1🔴, and Parvalbumin🟣. Monstrous endoplasmic reticulum in those Purkinje neuron dendrites! Credits @BrandonMeyerink #microscopymonday
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This work was the product of a super talented grad student @BrandonMeyerink. Brandon is on the Job Market for a #postdoc position. Any lab would be happy to have him and I am sad to see him leave. He has been a key player to get my lab up and running, and I am forever grateful.
Excited to share a new article from the lab, with grateful support from @SanfordResearch and @Dont4getMorgan!. BPAN is a devastating neurodegenerative #raredisease. Here, we characterize a mouse model mimicking a patient mutation. A thread.
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How are we following up on this? We are now collaborating with a @MayoClinic lab expert in 3D-EM, to further characterize those swollen BPAN neuron terminals, and we are dissecting the molecular mechanisms that are causing them.
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. In the brain, it appears that many Wdr45 KO axon terminals are massively swollen, presenting with spheroids. These spheroids are sometimes the size of several nuclei, they appear in all brain areas, and as soon as 14 days after birth for certain cells.
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The most striking takeaway from our BPAN model is that WDR45 loss impacts many neuron subtypes: Purkinje, dopaminergic, noradrenergic, and most, if not all, interneuron subtypes. Their cell bodies look totally normal (!), but their axon terminals are really messed up.
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This BPAN model shows vocalization defects in the first few days after birth, as well as long-lasting motor defects appearing after 3 months. Many defects are also seen in heterozygous females, which models the BPAN patient population most accurately.
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Excited to share a new article from the lab, with grateful support from @SanfordResearch and @Dont4getMorgan!. BPAN is a devastating neurodegenerative #raredisease. Here, we characterize a mouse model mimicking a patient mutation. A thread.
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RT @So_lets_kiLab70: It’s the final #MicroscopyMonday of the year—grateful to peer into the unknown, twiddle knobs myself, make discoveries….
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New preprint, marking a new direction for the lab. This work started with a 7:30 am call with a mom whose daughter was recently diagnosed with a rare disease @Dont4getMorgan #BPAN. We present phenotypes from a new mouse model mimicking a patient mutation.
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We did 3D electron microscopy of the deep cerebellar nuclei. What are these neatly organized stacks in what appears to be a presynaptic compartment? I honestly do not know. ⁉️ #cerebellum #purkinje
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RT @HiroshiKawasak7: 拡散希望Happy to share our new paper "Evolutionary changes leading to efficient glymphatic circulation in the mammalian br….
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The Pilaz Lab is moving. 50ft from our original place. We are expanding a bit. 🥹
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RT @So_lets_kiLab70: 🎆 #FluorescenceFriday vibes! Check out our new Staygold-PX fusion, targeting PI(4,5)P2 I imaged in neurons! 🌊 Lipids c….
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RT @ZoltanMolnar64: Please hold the date for the next CORTICAL DEVELOPMENT MEETING in Giardini Naxos, Sicily, Italy - May 25-28, 2025.@cort….
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