Liver and Chorion Cytochemistry

Overview

Journal J Inherit Metab Dis

Publisher Wiley

Date 1995 Jan 1

PMID 9053549

Citations 7

Authors

F Roels

B De Prest

G De Pestel

Affiliations

Soon will be listed here.

Abstract

Microscopic visualization of peroxisomes in chorionic villus cytotrophoblast and in biopsy and autopsy samples of liver and kidney, the presence of enlarged liver macrophages containing lipid droplets insoluble in acetone and n-hexane as well as polarizing inclusions formed by stacks of trilamellar sheets are of diagnostic value in peroxisomal disorders. Methods are presented for evaluating these structures by light microscopy; trilamellar inclusions are only detected by electron microscopy. Macrophage features are preserved in archival paraffin blocks. In adrenal cortex, insoluble lipid, polarizing inclusions and trilamellar structures should be looked for. The stains are easily reproducible, and all reagents are commercially available.

Citing Articles

Gene Trapped Mice: PMP34 Plays a Role in the Peroxisomal Degradation of Phytanic and Pristanic Acid.

Van Veldhoven P, de Schryver E, Young S, Zwijsen A, Fransen M, Espeel M Front Cell Dev Biol. 2020; 8:144.

PMID: 32266253 PMC: 7106852. DOI: 10.3389/fcell.2020.00144.

Peroxisomes in zebrafish: distribution pattern and knockdown studies.

Krysko O, Stevens M, Langenberg T, Fransen M, Espeel M, Baes M Histochem Cell Biol. 2010; 134(1):39-51.

PMID: 20556416 DOI: 10.1007/s00418-010-0712-z.

Phenylbutyrate up-regulates the adrenoleukodystrophy-related gene as a nonclassical peroxisome proliferator.

Gondcaille C, Depreter M, Fourcade S, Lecca M, Leclercq S, Martin P J Cell Biol. 2005; 169(1):93-104.

PMID: 15809314 PMC: 2171887. DOI: 10.1083/jcb.200501036.

A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parents.

Raas-Rothschild A, Wanders R, Mooijer P, Gootjes J, Waterham H, Gutman A Am J Hum Genet. 2002; 70(4):1062-8.

PMID: 11873320 PMC: 379104. DOI: 10.1086/339766.

A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency.

De Praeter CM , Gerwig G, BAUSE E, Nuytinck L, Vliegenthart J, Breuer W Am J Hum Genet. 2000; 66(6):1744-56.

PMID: 10788335 PMC: 1378052. DOI: 10.1086/302948.

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