What’s the FUS About? New ALS Mouse Model Thickens the Plot

RESEARCH NEWS 2014-02-14 Research News A single point mutation in the fused in sarcoma (FUS) gene can throw a wrench into the delicate mechanics of protecting and splicing genetic material, according to a new study. Reporting in the February 10 Journal of Clinical Investigation,

Maggie Pound

San Diego, United States

Zhenyu Yue on Unbiased screen for interactors of leucine-rich repeat kinase 2 supports a common pathway for sporadic and familial Parkinson disease.

COMMENT With increasing numbers of genetic risk factors for PD being identified, critical questions are whether these genes are connected, function in the same cellular pathways, or whether a common pathway exists for the majority of PD cases. Using large-scale s

Frédéric Checler on Lysosomal sorting of amyloid-β by the SORLA receptor is impaired by a familial Alzheimer's disease mutation.

COMMENT This is an important paper. It appears as one of very few examples of delineated risk factors for AD for which a plausible function linked to Aβ degradation is clearly documented. Of most interest, obviously, is the observation that an AD-linked mutation

Zhenyu Yue

Icahn School of Medicine at Mount Sinai
New York, United States

PAPER Vance C, Scotter EL, Nishimura AL, Troakes C, Mitchell JC, Kathe C, Urwin H, Manser C, Miller CC, Hortobágyi T, Dragunow M, Rogelj B, Shaw CE

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules.

Hum Mol Genet. 2013 Jul 1;22(13):2676-88. Epub 2013 Mar 7 PubMed: 23474818

Jonas Neher on Deficient neuron-microglia signaling results in impaired functional brain connectivity and social behavior.

COMMENT This impressive study from Cornelius Gross’ group again highlights the importance of appropriate immune surveillance during brain development. They show that an insufficient number of microglia in the developing CNS results in synaptic pruning deficits an

PAPER Zhan Y, Paolicelli RC, Sforazzini F, Weinhard L, Bolasco G, Pagani F, Vyssotski AL, Bifone A, Gozzi A, Ragozzino D, Gross CT

Deficient neuron-microglia signaling results in impaired functional brain connectivity and social behavior.

Nat Neurosci. 2014 Mar;17(3):400-6. Epub 2014 Feb 2 PubMed: 24487234

PAPER Neher JJ, Emmrich JV, Fricker M, Mander PK, Théry C, Brown GC

Phagocytosis executes delayed neuronal death after focal brain ischemia.

Proc Natl Acad Sci U S A. 2013 Oct 22;110(43):E4098-107. Epub 2013 Oct 7 PubMed: 24101459

SORLA Serves Up Aβ for Destruction

RESEARCH NEWS 2014-02-14 Research News The giant metal claw at a junkyard carries pieces of metal off to be crushed. Could SORLA  dispose of Aβ in a similar way? In the February 12 Science Translational Medicine, scientists led by Thomas Willnow, Max-Delbrück-Center for Molecular

PAPER Caglayan S, Takagi-Niidome S, Liao F, Carlo AS, Schmidt V, Burgert T, Kitago Y, Füchtbauer EM, Füchtbauer A, Holtzman DM, Takagi J, Willnow TE

Lysosomal sorting of amyloid-β by the SORLA receptor is impaired by a familial Alzheimer's disease mutation.

Sci Transl Med. 2014 Feb 12;6(223):223ra20. PubMed: 24523320

Jonas Neher

German Center for Neurodegenerative Diseases (DZNE)
Tuebingen , Germany

Tomas Falzone

CONICET
Duenos Aires, Argentina

Brendan Innes

Massachusetts General Hospital
Boston, United States

PAPER Kappeler L, De Magalhaes Filho C, Dupont J, Leneuve P, Cervera P, Périn L, Loudes C, Blaise A, Klein R, Epelbaum J, Le Bouc Y, Holzenberger M

Brain IGF-1 receptors control mammalian growth and lifespan through a neuroendocrine mechanism.

PLoS Biol. 2008 Oct 28;6(10):e254. PubMed: 18959478