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Peroxisomal Disorders

Known as: Peroxisomal Disorders [Disease/Finding], Peroxisomal Function Disorder, Peroxisomal Disorder 
A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be… Expand
National Institutes of Health

Related topics

Related topics

29 relations

Narrower (21)

Adrenoleukodystrophy, NeonatalHyperpipecolic AcidemiaPeroxisomal Dysfunction, GeneralPeroxisomal Dysfunction, Multiple
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In BloodInborn Errors of MetabolismMicrobiologicalchemically induced
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Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2018
Review
2018
Plasmalogen lipids: functional mechanism and their involvement in gastrointestinal cancer
The plasmalogens are a class of glycerophospholipids which contain a vinyl-ether and an ester bond at the sn-1 and sn-2 positions… Expand
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Highly Cited
2008
Highly Cited
2008
The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl–CoA esters
Peroxisomes play a major role in human cellular lipid metabolism, including the β‐oxidation of fatty acids. The most frequent… Expand
Highly Cited
2006
Highly Cited
2006
Chewing the fat: β-oxidation in signalling and development
Peroxisomal β-oxidation is involved not only in fatty acid catabolism and lipid housekeeping but also in metabolism of hormones… Expand
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Review
2004
Review
2004
Role of the adipocyte, free fatty acids, and ectopic fat in pathogenesis of type 2 diabetes mellitus: peroxisomal proliferator-activated receptor agonists provide a rational therapeutic approach.
Type 2 diabetes mellitus (T2DM) is characterized by insulin resistance in liver and muscle and impaired insulin secretion… Expand
Highly Cited
2004
Highly Cited
2004
A transgenic mouse with a deletion in the collagenous domain of adiponectin displays elevated circulating adiponectin and improved insulin sensitivity.
Adiponectin is a plasma protein expressed exclusively in adipose tissue. Adiponectin levels are linked to insulin sensitivity… Expand
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Review
2004
Review
2004
Peroxisomes, lipid metabolism, and peroxisomal disorders.
  • R. Wanders
  • Molecular genetics and metabolism
  • 2004
    • Corpus ID: 21895339
Peroxisomes catalyse a large variety of different cellular functions of which most have to do with lipid metabolism. This paper… Expand
Highly Cited
2000
Highly Cited
2000
Inactivation of the Peroxisomal Multifunctional Protein-2 in Mice Impedes the Degradation of Not Only 2-Methyl-branched Fatty Acids and Bile Acid Intermediates but Also of Very Long Chain Fatty Acids…
According to current views, peroxisomal β-oxidation is organized as two parallel pathways: the classical pathway that is… Expand
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Review
1998
Review
1998
Peroxisomal Disorders: Genotype, Phenotype, Major Neuropathologic Lesions, and Pathogenesis
Neurological dysfunction is a prominent feature of most peroxisomal disorders. Enormous progress in defining their gene defects… Expand
Highly Cited
1988
Highly Cited
1988
Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy.
We previously reported that in childhood adrenoleukodystrophy (C-ALD) and adrenomyeloneuropathy (AMN), the peroxisomal beta… Expand
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Highly Cited
1986
Highly Cited
1986
Peroxisomal alanine:glyoxylate aminotransferase deficiency in primary hyperoxaluria type I
Activities of alanine:glyoxylate aminotransferase in the livers of two patients with primary hyperoxaluria type I were… Expand
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