Complement is dispensable for neurodegeneration in Niemann-Pick disease type C

Journal of Neuroinflammation

Volumn 9, Issue , 2012, Pages

  Lopez, Manuel E ^a   Klein, Andres D ^a   Scott, Matthew P ^a  

^a HOWARD HUGHES MEDICAL INSTITUTE   (United States)

Author keywords

Complement; Extracellular matrix; Lysosomal storage disease; Microglia; Neurodegeneration; Niemann Pick; Purkinje neurons

Indexed keywords

COMPLEMENT COMPONENT C1Q; COMPLEMENT COMPONENT C1QA; COMPLEMENT COMPONENT C3; COMPLEMENT COMPONENT C3 RECEPTOR; COMPLEMENT COMPONENT C3A RECEPTOR; DIAMINOBENZIDINE; MATRIX METALLOPROTEINASE; PROTEOCHONDROITIN SULFATE; TOLL LIKE RECEPTOR; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; APOPTOSIS; ARTICLE; BRAIN CORTEX; BRAIN REGION; C3 GENE; CELL LOSS; CEREBELLUM; COMPLEMENT ACTIVATION; CONTROLLED STUDY; DEEP CEREBELLAR NUCLEI; EXTRACELLULAR MATRIX; GENE DELETION; HIPPOCAMPAL CA2 REGION; IMMUNOHISTOCHEMISTRY; MICROGLIA; MOUSE; NERVE DEGENERATION; NIEMANN PICK DISEASE; NONHUMAN; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; THALAMUS;

EID: 84866149223     PISSN: None     EISSN: 17422094     Source Type: Journal    
DOI: 10.1186/1742-2094-9-216     Document Type: Article

References (25)

1. Cluzeau CV, Watkins-Chow DE, Fu R, Borate B, Yanjanin N, Dail MK, Davidson CD, Walkley SU, Ory DS, Wassif CA, Pavan WJ, Porter FD. Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1. Hum Mol Genet 2012, 21:3632-3646. 10.1093/hmg/dds193, 22619379.
2. Lopez ME, Klein AD, Hong J, Dimbil UJ, Scott MP. Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C. Hum Mol Genet 2012, 21:2946-2960. 10.1093/hmg/dds126, 3373242, 22493001.
3. Stevens B, Allen NJ, Vazquez LE, Howell GR, Christopherson KS, Nouri N, Micheva KD, Mehalow AK, Huberman AD, Stafford B, Sher A, Litke AM, Lambris JD, Smith SJ, John SW, Barres BA. The classical complement cascade mediates CNS synapse elimination. Cell 2007, 131:1164-1178. 10.1016/j.cell.2007.10.036, 18083105.
4. Perry VH, O'Connor V. C1q: the perfect complement for a synaptic feast?. Nat Rev Neurosci 2008, 9:807-811. 10.1038/nrn2394, 18827829.
5. Paidassi H, Tacnet-Delorme P, Garlatti V, Darnault C, Ghebrehiwet B, Gaboriaud C, Arlaud GJ, Frachet P, Paidassi H, Tacnet-Delorme P, Garlatti V, Darnault C, Ghebrehiwet B, Gaboriaud C, Arlaud GJ, Frachet P. C1q binds phosphatidylserine and likely acts as a multiligand-bridging molecule in apoptotic cell recognition. J Immunol 2008, 180:2329-2338. 2632962, 18250442.
6. Liao G, Wen Z, Irizarry K, Huang Y, Mitsouras K, Darmani M, Leon T, Shi L, Bi X. Abnormal gene expression in cerebellum of Npc1-/- mice during postnatal development. Brain Res 2010, 1325:128-140. 2848886, 20153740.
7. Distl R, Kuban R, Komkov V, Kallwellis K, von Deimling A, Ohm TG. Differential gene expression in BALB/c Npc1-/- and Npc1+/+ cerebellum. NCBI GEO DataSets 2007, accession GSE5944..
8. Lobsiger CS, Boillee S, Cleveland DW. Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A 2007, 104:7319-7326. 10.1073/pnas.0702230104, 1863491, 17463094.
9. Ko DC, Milenkovic L, Beier SM, Manuel H, Buchanan J, Scott MP. Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease. PLoS Genet 2005, 1:81-95. 10.1371/journal.pgen.0010081, 1183526, 16103921.
10. Bellander BM, Singhrao SK, Ohlsson M, Mattsson P, Svensson M. Complement activation in the human brain after traumatic head injury. J Neurotrauma 2001, 18:1295-1311. 10.1089/08977150152725605, 11780861.
11. Zhao M, Choi YS, Obrietan K, Dudek SM. Synaptic plasticity (and the lack thereof) in hippocampal CA2 neurons. J Neurosci 2007, 27:12025-12032. 10.1523/JNEUROSCI.4094-07.2007, 17978044.
12. Foscarin S, Ponchione D, Pajaj E, Leto K, Gawlak M, Wilczynski GM, Rossi F, Carulli D. Experience-dependent plasticity and modulation of growth regulatory molecules at central synapses. PLoS One 2011, 6:e16666. 10.1371/journal.pone.0016666, 3031615, 21304956.
13. Kirschfink M, Blase L, Engelmann S, Schwartz-Albiez R. Secreted chondroitin sulfate proteoglycan of human B cell lines binds to the complement protein C1q and inhibits complex formation of C1. J Immunol 1997, 158:1324-1331.
14. Ilieva H, Polymenidou M, Cleveland DW. Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol 2009, 187:761-772. 10.1083/jcb.200908164, 2806318, 19951898.
15. Sarna JR, Larouche M, Marzban H, Sillitoe RV, Rancourt DE, Hawkes R. Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease. J Comp Neurol 2003, 456:279-291. 10.1002/cne.10522, 12528192.
16. Sillitoe RV, Vogel MW, Joyner AL. Engrailed homeobox genes regulate establishment of the cerebellar afferent circuit map. J Neurosci 2010, 30:10015-10024. 10.1523/JNEUROSCI.0653-10.2010, 2921890, 20668186.
17. Lopez ME, Klein AD, Dimbil UJ, Scott MP. Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder. J Neurosci 2011, 31:4367-4378. 10.1523/JNEUROSCI.5981-10.2011, 3071647, 21430138.
18. Lund JM, Alexopoulou L, Sato A, Karow M, Adams NC, Gale NW, Iwasaki A, Flavell RA. Recognition of single-stranded RNA viruses by Toll-like receptor 7. Proc Natl Acad Sci U S A 2004, 101:5598-5603. 10.1073/pnas.0400937101, 397437, 15034168.
19. Tabeta K, Hoebe K, Janssen EM, Du X, Georgel P, Crozat K, Mudd S, Mann N, Sovath S, Goode J, Shamel L, Herskovits AA, Portnoy DA, Cooke M, Tarantino LM, Wiltshire T, Steinberg BE, Grinstein S, Beutler B. The Unc93b1 mutation 3d disrupts exogenous antigen presentation and signaling via Toll-like receptors 3, 7 and 9. Nat Immunol 2006, 7:156-164.
20. Shipley JM, Wesselschmidt RL, Kobayashi DK, Ley TJ, Shapiro SD. Metalloelastase is required for macrophage-mediated proteolysis and matrix invasion in mice. Proc Natl Acad Sci U S A 1996, 93:3942-3946. 10.1073/pnas.93.9.3942, 39464, 8632994.
21. Bouillet P, Metcalf D, Huang DC, Tarlinton DM, Kay TW, Kontgen F, Adams JM, Strasser A. Proapoptotic Bcl-2 relative Bim required for certain apoptotic responses, leukocyte homeostasis, and to preclude autoimmunity. Science 1999, 286:1735-1738. 10.1126/science.286.5445.1735, 10576740.
22. Bouillet P, Robati M, Adams JM, Strasser A. Loss of pro-apoptotic BH3-only Bcl-2 family member Bim does not protect mutant Lurcher mice from neurodegeneration. J Neurosci Res 2003, 74:777-781. 10.1002/jnr.10805, 14635229.
23. Depboylu C, Schorlemmer K, Klietz M, Oertel WH, Weihe E, Hoglinger GU, Schafer MK. Upregulation of microglial C1q expression has no effects on nigrostriatal dopaminergic injury in the MPTP mouse model of Parkinson disease. J Neuroimmunol 2011, 236:39-46. 10.1016/j.jneuroim.2011.05.006, 21640391.
24. Botto M, Dell'Agnola C, Bygrave AE, Thompson EM, Cook HT, Petry F, Loos M, Pandolfi PP, Walport MJ. Homozygous C1q deficiency causes glomerulonephritis associated with multiple apoptotic bodies. Nat Genet 1998, 19:56-59. 10.1038/ng0598-56, 9590289.
25. Wessels MR, Butko P, Ma M, Warren HB, Lage AL, Carroll MC. Studies of group B streptococcal infection in mice deficient in complement component C3 or C4 demonstrate an essential role for complement in both innate and acquired immunity. Proc Natl Acad Sci U S A 1995, 92:11490-11494. 10.1073/pnas.92.25.11490, 40427, 8524789.