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Peroxisome synthesis in the absence ...

South, Sarah Thornell.

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Peroxisome synthesis in the absence of preexisting peroxisomes.

紀錄類型:	書目-語言資料,印刷品 : Monograph/item
正題名/作者:	Peroxisome synthesis in the absence of preexisting peroxisomes./
作者:	South, Sarah Thornell.
面頁冊數:	112 p.
附註:	Adviser: Stephen J. Gould.
Contained By:	Dissertation Abstracts International62-10B.
標題:	Biology, Cell. -
電子資源:	http://pqdd.sinica.edu.tw/twdaoapp/servlet/advanced?query=3028332
ISBN:	0493405011

Peroxisome synthesis in the absence of preexisting peroxisomes.
South, Sarah Thornell.

Peroxisome synthesis in the absence of preexisting peroxisomes. - 112 p.

Adviser: Stephen J. Gould.

Thesis (Ph.D.)--The Johns Hopkins University, 2002.

Peroxisomes are single membrane bound organelles involved in diverse metabolic functions. Defects in peroxisome biogenesis lead to a group of autosomal recessive diseases known as the Peroxisome Biogenesis Disorders (PBDs). Most PBD patients have a defect restricted to the import of peroxisomal matrix proteins and therefore their cells still contain peroxisome membrane remnants. However, I have identified three PBD patients with the unusual cellular phenotype of lacking any identifiable peroxisome membrane structures. In the process of determining the genetic defects of these patients I discovered <italic> PEX16</italic>, a new human gene involved in peroxisome membrane biogenesis. I determined that one of these patients was homozygous for an inactivating mutation in <italic>PEX16</italic> and the other two patients were each homozygous for inactivating mutations in <italic>PEX3</italic>. Characterization of PEX3 and PEX16 revealed they are required for either peroxisome membrane biogenesis or peroxisome membrane protein import and are themselves integral peroxisomal membrane proteins. Expression of either <italic>PEX16</italic> or <italic> PEX3</italic> cDNA in their respectively deficient patient cell lines restores functional peroxisomes, demonstrating that peroxisomes can form in the absence of preexisting peroxisomes.

ISBN: 0493405011Subjects--Topical Terms:

1017686
Biology, Cell.

Peroxisome synthesis in the absence of preexisting peroxisomes.
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245 1 0 $a Peroxisome synthesis in the absence of preexisting peroxisomes.
300 $a 112 p.
500 $a Adviser: Stephen J. Gould.
500 $a Source: Dissertation Abstracts International, Volume: 62-10, Section: B, page: 4325.
502 $a Thesis (Ph.D.)--The Johns Hopkins University, 2002.
520 $a Peroxisomes are single membrane bound organelles involved in diverse metabolic functions. Defects in peroxisome biogenesis lead to a group of autosomal recessive diseases known as the Peroxisome Biogenesis Disorders (PBDs). Most PBD patients have a defect restricted to the import of peroxisomal matrix proteins and therefore their cells still contain peroxisome membrane remnants. However, I have identified three PBD patients with the unusual cellular phenotype of lacking any identifiable peroxisome membrane structures. In the process of determining the genetic defects of these patients I discovered <italic> PEX16</italic>, a new human gene involved in peroxisome membrane biogenesis. I determined that one of these patients was homozygous for an inactivating mutation in <italic>PEX16</italic> and the other two patients were each homozygous for inactivating mutations in <italic>PEX3</italic>. Characterization of PEX3 and PEX16 revealed they are required for either peroxisome membrane biogenesis or peroxisome membrane protein import and are themselves integral peroxisomal membrane proteins. Expression of either <italic>PEX16</italic> or <italic> PEX3</italic> cDNA in their respectively deficient patient cell lines restores functional peroxisomes, demonstrating that peroxisomes can form in the absence of preexisting peroxisomes.
520 $a The mechanism by which PEX3 and PEX16 induce the formation of new peroxisomes is unclear. Current hypotheses on the origin of cellular organelles state that organelles always originate from preexisting organelles. In explaining the formation of new peroxisomes in the absence of preexisting peroxisomes, it has been suggested that PEX3 and PEX16 enter the endoplasmic reticulum (ER) and promote the budding of preperoxisomal vesicles that then matures into functional peroxisomes. In order to test this hypothesis we individually examined <italic>PEX3</italic>- and <italic>PEX16</italic>-mediated peroxisome synthesis in human cells inhibited at multiple steps of vesicle trafficking from the ER as well as <italic>PEX3</italic>-mediated peroxisome synthesis in <italic>Saccharomyces cerevisiae</italic> lacking a function ER translocation apparatus. These studies demonstrate that <italic>PEX16</italic>- and <italic> PEX3</italic>-mediated peroxisome synthesis does not involve protein trafficking through the ER and leads to the possibility that PEX3 and PEX16 may be involved in <italic>de novo</italic> peroxisome synthesis. Therefore we propose that peroxisomes can form either by the division of preexisting peroxisomes or through <italic>PEX3</italic>- and <italic>PEX16</italic>-mediated formation of new peroxisomes.
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