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Pex11b  -  peroxisomal biogenesis factor 11 beta

Mus musculus

Synonyms: PEX11-beta, PEX11beta, Peroxin-11B, Peroxisomal biogenesis factor 11B, Peroxisomal membrane protein 11B, ...
 
 
 
 
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Disease relevance of Pex11b

  • Here we show that mice lacking peroxisomal membrane protein PEX11 beta display several pathologic features shared by these mouse models of Zellweger syndrome, including neuronal migration defects, enhanced neuronal apoptosis, a developmental delay, hypotonia, and neonatal lethality [1].
 

High impact information on Pex11b

  • We also observed that mouse cells lacking PEX11beta display reduced peroxisome abundance, even in the absence of peroxisomal metabolic substrates, and that PEX11beta(-/-) mice are partially deficient in two distinct peroxisomal metabolic pathways, ether lipid synthesis and very long chain fatty acid oxidation [2].

References

  1. PEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome function. Li, X., Baumgart, E., Morrell, J.C., Jimenez-Sanchez, G., Valle, D., Gould, S.J. Mol. Cell. Biol. (2002) [Pubmed]
  2. PEX11 promotes peroxisome division independently of peroxisome metabolism. Li, X., Gould, S.J. J. Cell Biol. (2002) [Pubmed]
 
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