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PMID:19933170

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Citation

Chen, H, Liu, Z and Huang, X (2010) Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism. Hum. Mol. Genet. 19:494-505

Abstract

Peroxisomes are vital eukaryotic organelles that participate in lipid metabolism, in particular the metabolism of very-long-chain fatty acids (VLCFA). The biogenesis of peroxisomes is regulated by a set of peroxin proteins (PEX). In humans, mutations affecting peroxin protein production or function result in devastating diseases classified as peroxisome biogenesis disorders (PBD). The way in which peroxisomal dysfunction leads to the pathophysiological consequences of PBD is not well understood. Here we report that Drosophila pex mutants faithfully recapitulate several key features of human PBD, including impaired peroxisomal protein import, elevated VLCFA levels and growth retardation. Moreover, disruption of pex function results in spermatogenesis defects, including spermatocyte cytokinesis failure in Drosophila. Importantly, increased VLCFA levels enhance these spermatogenesis defects whereas reduced VLCFA levels alleviate them. Thus, regulation of proper VLCFA levels by pex genes is crucial for spermatogenesis. Together our study reveals an indispensable function of pex genes during spermatogenesis and provides a causative link between the phenotypic severity of pex mutants and VLCFA levels.

Links

PubMed Online version:10.1093/hmg/ddp518

Keywords

Animals; Disease Models, Animal; Drosophila/genetics; Drosophila/growth & development; Drosophila/metabolism; Drosophila Proteins/genetics; Drosophila Proteins/metabolism; Fatty Acids/chemistry; Fatty Acids/metabolism; Humans; Male; Peroxisomal Disorders/genetics; Peroxisomal Disorders/metabolism; Spermatocytes/growth & development; Spermatocytes/metabolism; Spermatogenesis

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

DROME:Q7JRD4

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q7K1R0

involved_in

GO:0016558: protein import into peroxisome matrix

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q7K1R0

involved_in

GO:0007286: spermatid development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q7K1R0

involved_in

GO:0007285: primary spermatocyte growth

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q7K1R0

involved_in

GO:0007112: male meiosis cytokinesis

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q7K1R0

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q95SJ3

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VPB9

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:PEX12

involved_in

GO:0007286: spermatid development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:PEX12

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VSH8

involved_in

GO:0016558: protein import into peroxisome matrix

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VSH8

involved_in

GO:0007286: spermatid development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VSH8

involved_in

GO:0007285: primary spermatocyte growth

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VSH8

involved_in

GO:0007112: male meiosis cytokinesis

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VSH8

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VUC7

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

involved_in

GO:0016558: protein import into peroxisome matrix

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

involved_in

GO:0007286: spermatid development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

involved_in

GO:0007285: primary spermatocyte growth

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

involved_in

GO:0007112: male meiosis cytokinesis

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

NOT|involved_in

GO:0045046: protein import into peroxisome membrane

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9W0D7

involved_in

GO:0042760: very long-chain fatty acid catabolic process

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9Y090

involved_in

GO:0048137: spermatocyte division

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete


See also

References

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