Organelle biogenesis: en BLOC exchange for RAB32 and RAB38

Curr Biol. 2012 Nov 20;22(22):R963-5. doi: 10.1016/j.cub.2012.10.005.

Abstract

Prominent subtypes of the genetic disorder Hermansky-Pudlak syndrome result from defects in a mysterious protein complex, BLOC-3. New work identifies BLOC-3 as a guanine nucleotide exchange factor for two RAB GTPases previously implicated in lysosome-related organelle biogenesis.

MeSH terms

  • Animals
  • Cell Line, Tumor
  • Gene Expression Regulation
  • Guanine Nucleotide Exchange Factors / genetics
  • Guanine Nucleotide Exchange Factors / metabolism
  • Hermanski-Pudlak Syndrome / genetics
  • Hermanski-Pudlak Syndrome / metabolism
  • Humans
  • Melanocytes / metabolism
  • Mice
  • Multiprotein Complexes / genetics
  • Multiprotein Complexes / metabolism
  • Organelles / physiology*
  • Protein Transport
  • Rats
  • rab GTP-Binding Proteins / genetics
  • rab GTP-Binding Proteins / metabolism*

Substances

  • Guanine Nucleotide Exchange Factors
  • Multiprotein Complexes
  • rab GTP-Binding Proteins