Peroxisomal disorders

Gerald Raymond

Research output: Contribution to journalReview article

8 Citations (Scopus)

Abstract

Peroxisomes are membrane-bound subcellular organelles that are involved in a variety of cellular functions. Disorders of peroxisomes, either in their assembly or single enzyme deficiencies, manifest themselves in the nervous system both in development and later in life. Most peroxisomal matrix proteins are targeted using one of the targeting sequences, whereas integral peroxisomal membrane proteins employ a different method. Peroxisomal importation is unique, allowing the importation of oligomerized proteins, and uses a specific extended shuttle system of receptor and cargo. The understanding of peroxisomal assembly is important because peroxisomal biogenesis disorders such as Zellweger syndrome result from these defects, and the resulting failure causes widespread deficiencies in peroxisomal biochemical function, X-linked adrenoleukodystrophy, representing the other group of peroxisomal disorders, is caused by the lack of the adrenoleukodystrophy protein, with an accumulation of very long chain fatty acids. New information on clinical incidence, phenotypic variability, and pathogenesis is becoming available and will have implications for possible therapies.

Original languageEnglish (US)
Pages (from-to)783-787
Number of pages5
JournalCurrent Opinion in Neurology
Volume14
Issue number6
DOIs
StatePublished - Dec 1 2001

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Peroxisomal Disorders
Adrenoleukodystrophy
Peroxisomes
Zellweger Syndrome
Proteins
Organelles
Nervous System
Membrane Proteins
Fatty Acids
Membranes
Incidence
Enzymes
Therapeutics

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

Raymond, Gerald. / Peroxisomal disorders. In: Current Opinion in Neurology. 2001 ; Vol. 14, No. 6. pp. 783-787.
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Peroxisomal disorders. / Raymond, Gerald.

In: Current Opinion in Neurology, Vol. 14, No. 6, 01.12.2001, p. 783-787.

Research output: Contribution to journalReview article

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