Literature DB >> 10863942

Disorders related to peroxisomal membranes.

J Gärtner1.   

Abstract

The peroxisome is a ubiquitous, subcellular organelle participating in a diverse array of metabolic pathways. The peroxisomal membrane and its components play a key role in organelle assembly and functions. Disorders related to peroxisomal membranes are the peroxisome biogenesis disorders and X-linked adrenoleukodystrophy. Identification and functional characterization of these disease genes is proceeding at rapid pace helped immeasurably by work in various yeast model systems. The ultimate goal is to elucidate how the encoded proteins interact to produce apparently normal and functioning peroxisomes. The achievement of this goal will lead to a better understanding of disease pathogenesis and hopefully open therapeutic options.

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Year:  2000        PMID： 10863942     DOI： 10.1023/a:1005636113499

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  37 in total

1.  Peroxisomal ABC transporters.

Authors:  N Shani; D Valle
Journal:  Methods Enzymol       Date:  1998       Impact factor: 1.600

Review 2.  Clinical and genetic aspects of X-linked adrenoleukodystrophy.

Authors:  J Gärtner; A Braun; A Holzinger; P Roerig; H G Lenard; A A Roscher
Journal:  Neuropediatrics       Date:  1998-02       Impact factor: 1.947

Review 3.  Biogenesis of peroxisomes.

Authors:  P B Lazarow; Y Fujiki
Journal:  Annu Rev Cell Biol       Date:  1985

4.  Identification of a common PEX1 mutation in Zellweger syndrome.

Authors:  C S Collins; S J Gould
Journal:  Hum Mutat       Date:  1999       Impact factor: 4.878

5.  The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor.

Authors:  T Yahraus; N Braverman; G Dodt; J E Kalish; J C Morrell; H W Moser; D Valle; S J Gould
Journal:  EMBO J       Date:  1996-06-17       Impact factor: 11.598

6.  Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor.

Authors:  A M Motley; E H Hettema; E M Hogenhout; P Brites; A L ten Asbroek; F A Wijburg; F Baas; H S Heijmans; H F Tabak; R J Wanders; B Distel
Journal:  Nat Genet       Date:  1997-04       Impact factor: 38.330

7.  Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins.

Authors:  L T Braiterman; S Zheng; P A Watkins; M T Geraghty; G Johnson; M C McGuinness; A B Moser; K D Smith
Journal:  Hum Mol Genet       Date:  1998-02       Impact factor: 6.150

8.  Gene redundancy and pharmacological gene therapy: implications for X-linked adrenoleukodystrophy.

Authors:  S Kemp; H M Wei; J F Lu; L T Braiterman; M C McGuinness; A B Moser; P A Watkins; K D Smith
Journal:  Nat Med       Date:  1998-11       Impact factor: 53.440

9.  Peroxisome assembly factor-2, a putative ATPase cloned by functional complementation on a peroxisome-deficient mammalian cell mutant.

Authors:  T Tsukamoto; S Miura; T Nakai; S Yokota; N Shimozawa; Y Suzuki; T Orii; Y Fujiki; F Sakai; A Bogaki; H Yasumo; T Osumi
Journal:  Nat Genet       Date:  1995-12       Impact factor: 38.330

10.  The sorting sequence of the peroxisomal integral membrane protein PMP47 is contained within a short hydrophilic loop.

Authors:  J M Dyer; J A McNew; J M Goodman
Journal:  J Cell Biol       Date:  1996-04       Impact factor: 10.539
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  1 in total

1.  The Arabidopsis pxa1 mutant is defective in an ATP-binding cassette transporter-like protein required for peroxisomal fatty acid beta-oxidation.

Authors:  B K Zolman; I D Silva; B Bartel
Journal:  Plant Physiol       Date:  2001-11       Impact factor: 8.340
  1 in total

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