GONUTS has been updated to MW1.31 Most things seem to be working but be sure to report problems.

Have any questions? Please email us at ecoliwiki@gmail.com

PMID:11792727

From GONUTS
Jump to: navigation, search
Citation

Hogenboom, S, Romeijn, GJ, Houten, SM, Baes, M, Wanders, RJ and Waterham, HR (2002) Absence of functional peroxisomes does not lead to deficiency of enzymes involved in cholesterol biosynthesis. J. Lipid Res. 43:90-8

Abstract

To unravel the conflicting data concerning the dependence of human cholesterol biosynthesis on functional peroxisomes, we determined activities and levels of selected enzymes involved in cholesterol biosynthesis in livers of PEX5 knockout mice, a well-characterized model for human Zellweger syndrome. We found that all enzymes measured, including putative peroxisomal enzymes, are at least as active in the peroxisome-deficient Zellweger mice as in control mice, indicating that mislocalization of enzymes to the cytosol does not lead to decreased activity or degradation. Prompted by these results, we re-examined this aspect in human subjects by specific enzyme activity measurements and immunoblotting with highly specific antisera. Our results show that the previously reported deficiencies of mevalonate kinase and phosphomevalonate kinase activity in livers from human Zellweger patients reflect the bad condition of the livers, rather than mislocalization to the cytosol. Our data provide an explanation for the conflicting findings in the literature and show that great care should be taken in the interpretation of data obtained in postmortem material.

Links

PubMed

Keywords

Animals; Carboxy-Lyases/metabolism; Cholesterol/biosynthesis; Disease Models, Animal; Enzyme Activation; Enzyme Stability; Humans; Hydroxymethylglutaryl CoA Reductases/metabolism; Immunoblotting/methods; Liver/enzymology; Mice; Peroxisomes/metabolism; Phosphotransferases (Alcohol Group Acceptor)/metabolism; Phosphotransferases (Phosphate Group Acceptor)/metabolism; Zellweger Syndrome/metabolism

Significance

Annotations

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

MOUSE:PEX5

acts_upstream_of_or_within

GO:0006625: protein targeting to peroxisome

ECO:0000304: author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

MOUSE:PEX5

enables

GO:0000268: peroxisome targeting sequence binding

ECO:0000304: author statement supported by traceable reference used in manual assertion

F

Seeded From UniProt

complete

MOUSE:PERM

enables

GO:0004601: peroxidase activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:CATA

enables

GO:0016684: oxidoreductase activity, acting on peroxide as acceptor

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:DHE3

enables

GO:0004353: glutamate dehydrogenase [NAD(P)+] activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

HUMAN:MVD1

involved_in

GO:0008299: isoprenoid biosynthetic process

ECO:0000314: direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:MVD1

enables

GO:0004163: diphosphomevalonate decarboxylase activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:GNPAT

enables

GO:0016287: glycerone-phosphate O-acyltransferase activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:HMDH

enables

GO:0004420: hydroxymethylglutaryl-CoA reductase (NADPH) activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:PMVK

enables

GO:0004631: phosphomevalonate kinase activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:KIME

enables

GO:0004496: mevalonate kinase activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

MOUSE:DHCR7

enables

GO:0047598: 7-dehydrocholesterol reductase activity

ECO:0000314: direct assay evidence used in manual assertion

F

Seeded From UniProt

complete


See also

References

See Help:References for how to manage references in GONUTS.