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Restoration of peroxisome biogenesis in a peroxisome‐deficient mammalian cell line by expression of either the 35 kDa or the 70 kDa peroxisomal membrane proteins
Author(s) -
Gärtner J.,
Obie C.,
Watkins P.,
Valle D.
Publication year - 1994
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf00711820
Subject(s) - medical school , peroxisomal disorder , medicine , peroxisome , library science , gerontology , medical education , computer science , receptor
Peroxisomes are single-membrane-bound organelles present in nearly all eukaryotic cells. Their size and number vary depending on the type of cell and its physiological state. The organelle matrix contains more than 40 enzymes that catalyse a variety of anabolic and catabolic reactions. Both the 35kDa (PMP35) and 70kDa (PMP70) peroxisomal membrane proteins are found only in the peroxisomal membrane and appear to be required for peroxisome assembly. PMP70 is a member of the ATP-binding cassette transporter family, while PMP35 has a zinc-finger domain (Gartner et al 1992; Shimozawa et al 1992). Peroxisomal biogenesis involves import of newly synthesized membrane and matrix proteins into preexisting peroxisomes followed by growth and fission of the organelle