Huang, Eric

<p>Nov 11, 2016 06:25 PM &nbsp;MD, PhD &nbsp;neuropathology</p>

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"we see 150 brains per year at UCSF &nbsp;(at autopsy)</p>

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Frontotemporal lobar degeneration (FTLD) is the second most common neurodegenerative disease in patients younger than 60 years old. A significant number of FTLD patients develop motor neuron degeneration with cellular and molecular signatures similar to those seen in patients with amyotrophic lateral sclerosis (ALS).&nbsp;</p>

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To understand mechanisms that connect these two devastating diseases, we focus on genes in which mutations have been causally linked to human diseases.&nbsp;</p>

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Specifically, our results show that<b> FTLD gene Progranulin (PGRN) is required to SUPPRESS neuroinflammation in injury or toxin-induced neurodegenerative conditions</b>.</p>

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<b> Loss-of-function in PGRN promotes neurodegeneration through activation of microgliosis and increases in proinflammatory cytokines.</b>&nbsp;</p>

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Our current effort focuses on how PGRN deficiency causes both cell autonomous and non-cell autonomous defects leading to neurodegeneration in the aging process. Finally, we are interested in characterizing the similarities and differences among three different ALS mouse models, ALS-SOD1, ALS-FUS and ALS-TDP43. Our efforts to compare and contrast these different models should provide new insights to the pathogenesis and therapeutics for both familial and sporadic ALS.

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