Tuesday, October 2, 2012

Inventory of fly peroxisomal proteins. Links to diseases. Recent report.

This recent paper catalogs fly peroxisomal proteins.

Faust JE, Verma A, Peng C, McNew JA. An Inventory of Peroxisomal Proteins and Pathways in Drosophila melanogaster. Traffic. 2012 Oct;13(10):1378-92. doi: 10.1111/j.1600-0854.2012.01393.x. PubMed PMID: 22758915; PubMed Central PMCID: PMC3443258.

Disruption of peroxisomal-related factors in humans can cause peroxisomal biogenesis disorders such as Zellweger syndrome. Additional relevant disease terms include peroxisomal acyl-CoA oxidase deficiency, rhizomelic chondrodysplasia punctata, Refsum disease, and neonatal adrenoleukodystrophy. You can read about peroxisomal biogenesis disorders at Gene Reviews (includes information about human disease-associated genes).

Evolutionary conservation? The answer appears to be yes. From the paper, "We have identified all of the major vertebrate peroxisomal pathways in Drosophila."

At least these three papers discuss fly models of peroxisomal biogenesis disorders.

Mast FD, Li J, Virk MK, Hughes SC, Simmonds AJ, Rachubinski RA. A Drosophila model for the Zellweger spectrum of peroxisome biogenesis disorders. Dis Model Mech. 2011 Sep-Oct;4(5):659-72. doi: 10.1242/dmm.007419. PubMed PMID: 21669930; PubMed Central PMCID: PMC3180231.

Nakayama M, Sato H, Okuda T, Fujisawa N, Kono N, Arai H, Suzuki E, Umeda M, Ishikawa HO, Matsuno K. Drosophila carrying pex3 or pex16 mutations are models of Zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes. PLoS One. 2011;6(8):e22984. PubMed PMID: 21826223; PubMed Central PMCID: PMC3149631.

Chen H, Liu Z, Huang X. Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism. Hum Mol Genet. 2010 Feb 1;19(3):494-505. PubMed PMID: 19933170.

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