Biochemistry of Peroxisomes

Subcellular localisation and processing of non‐specific lipid transfer protein are not aberrant in Rhizomelic Chondrodysplasia Punctata fibroblasts

“…Sequence comparison revealed a striking homology between the amino acid sequence upstream of the processing site of nsLTP and 3-0xoacyE CoA thiolase [2,33], suggesting that a single peroxisomal protease is responsible for the peroxisomal processing of both proteins. If this is indeed the case, it can be excluded that a mutation in a gene encoding a peroxisomal protease is responsible for the deficiency in the import of 3-oxoacyl-CoA thiolase in RCDP patients.…”

“…Peroxisomes, which are subcellular organelles first characterised biochemically in the 1960's by De Duvc and co-workers [l], contain enzymes involved in a number of important metabolic processes such as the @-oxidation of very-long-chain fatty acids and the biosynthesis of ether phospholipids (reviewed in [2]). In recent years various inherited diseases in man have been described in which one or several peroxisomal functions are impaired [3-81. In one category of such diseases, exemplified by the cerebra-hepato-renal (Zellweger) syndrome (ZS), morphologically distinguishable peroxisomes are decreased in number or even absent and there is a generalised loss of peroxisomal functions [3-61.…”

“…in both these respects the behaviour of nsLTP resembles that of 3-oxoacyl-CoA thiolase. Since there is a striking homology in the amino acid sequence upstream of the processing site in the two proteins (see [2,33]), a single peroxisomal protease 'may be responsible for the processing of both proteins. Furthermore, these facts suggest that the protease is deficient in cells lacking peroxisomes.…”

Peroxisomes: simple in function but complex in maintenance

“…In S. cerevisiae, fatty acid oxidation takes place only in peroxisomes 9 . In mammalian cells, shortened fatty acids are exported from peroxisomes to mitochondria for complete degradation 10 . The absence of mitochondrial isoenzymes for ␤-oxidation means that acetyl-CoA and NADH are produced in yeast peroxisomes (Fig.…”

The Peroxisomal Catalase Gene in the Methylotrophic YeastPichia methanolica

“…3,4) and the physiological importance of catalase activity in the peroxisome is highlighted by the existence of the genetic disease Acatalasemia. 5) In view of these facts, catalase is one of the most important enzymes involved in peroxisomal oxidative metabolism.…”