Literature DB >> 6415912

Striated adrenocortical cells in cerebro-hepato-renal (Zellweger) syndrome.

S Goldfischer, J M Powers, A B Johnson, S Axe, F R Brown, H W Moser.   

Abstract

Adrenal glands from eight patients with the cerebro-hepato-renal syndrome, a disease in which there are no morphologically demonstrable peroxisomes, were studied histologically; one of the eight was also examined ultrastructurally. Seven of the eight demonstrated striated adrenocortical cells in the inner portion of the adrenal cortex. Ultrastructural examination confirmed that the striated cells contained the lammellae and lamellar-lipid profiles of very long chain fatty acids-cholesterol esters that are characteristic of adreno-leukodystrophy. This morphologic observation further emphasizes the common pathogenetic features of the cerebro-hepato-renal (Zellweger) syndrome and adreno-leukodystrophy.

Entities:  

Mesh：

Year:  1983        PMID： 6415912     DOI： 10.1007/BF00734851

Source DB:  PubMed          Journal:  Virchows Arch A Pathol Anat Histopathol        ISSN： 0174-7398


  24 in total

1.  Cerebro-hepato-renal syndrome of Zellweger. A report of eight cases with comments upon the incidence, the liver lesion, and a fault in pipecolic acid metabolism.

Authors:  D M Danks; P Tippett; C Adams; P Campbell
Journal:  J Pediatr       Date:  1975-03       Impact factor: 4.406

2.  The adrenal cortex in adreno-leukodystrophy.

Authors:  J M Powers; H H Schaumburg
Journal:  Arch Pathol       Date:  1973-11

3.  Cerebro-hepato-renal syndrome of Zellweger: an inherited disorder of neuronal migration.

Authors:  J J Volpe; R D Adams
Journal:  Acta Neuropathol       Date:  1972       Impact factor: 17.088

4.  The role of peroxisomes in the response of the toad bladder to aldosterone.

Authors:  R G Jones; W L Davis; D B Goodman
Journal:  Ann N Y Acad Sci       Date:  1982       Impact factor: 5.691

Review 5.  Fetal adrenoleukodystrophy: the significance of pathologic lesions in adrenal gland and testis.

Authors:  J M Powers; H W Moser; A B Moser; H H Schaumburg
Journal:  Hum Pathol       Date:  1982-11       Impact factor: 3.466

6.  Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids.

Authors:  F R Brown; A J McAdams; J W Cummins; R Konkol; I Singh; A B Moser; H W Moser
Journal:  Johns Hopkins Med J       Date:  1982-12

7.  Neonatal-onset adrenoleukodystrophy in a girl.

Authors:  J E Haas; E S Johnson; D L Farrell
Journal:  Ann Neurol       Date:  1982-11       Impact factor: 10.422

8.  Neonatal adrenoleukodystrophy: clinical, pathologic, and biochemical delineation of a syndrome affecting both males and females.

Authors:  R Jaffe; P Crumrine; Y Hashida; H W Moser
Journal:  Am J Pathol       Date:  1982-07       Impact factor: 4.307

9.  New phenotypic variant of adrenoleukodystrophy. Pathologic, ultrastructural, and biochemical study in two brothers.

Authors:  H J Manz; M Schuelein; D C McCullough; Y Kishimoto; R M Eiben
Journal:  J Neurol Sci       Date:  1980-03       Impact factor: 3.181

10.  [Morphology and diagnosis of Zellweger syndrome. A contribution to combined cytochemical-finestructural identification of peroxisomes in autopsy material and frozen liver tissue with case report].

Authors:  J Müller-Höcker; K Bise; W Endres; G Hübner
Journal:  Virchows Arch A Pathol Anat Histol       Date:  1981
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  12 in total

Review 1.  Zellweger syndrome and associated phenotypes.

Authors:  D R FitzPatrick
Journal:  J Med Genet       Date:  1996-10       Impact factor: 6.318

2.  A pathological study of a peripheral nerve in a case of neonatal adrenoleukodystrophy.

Authors:  T Mito; K Takada; S Akaboshi; S Takashima; K Takeshita; Y Origuchi
Journal:  Acta Neuropathol       Date:  1989       Impact factor: 17.088

3.  Neonatal adrenoleukodystrophy.

Authors:  P Aubourg; J Scotto; F Rocchiccioli; D Feldmann-Pautrat; O Robain
Journal:  J Neurol Neurosurg Psychiatry       Date:  1986-01       Impact factor: 10.154

Review 4.  Peroxisomal disorders: clinical characterization.

Authors:  L Monnens; H Heymans
Journal:  J Inherit Metab Dis       Date:  1987       Impact factor: 4.982

Review 5.  The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction.

Authors:  P L Faust; H M Su; A Moser; H W Moser
Journal:  J Mol Neurosci       Date:  2001 Apr-Jun       Impact factor: 3.444

Review 6.  Peroxisomal disorders: a newly recognised group of genetic diseases.

Authors:  R B Schutgens; H S Heymans; R J Wanders; H van den Bosch; J M Tager
Journal:  Eur J Pediatr       Date:  1986-02       Impact factor: 3.183

7.  Disturbed adrenocortical function in cerebro-hepato-renal syndrome of Zellweger.

Authors:  L Govaerts; L Monnens; T Melis; F Trijbels
Journal:  Eur J Pediatr       Date:  1984-11       Impact factor: 3.183

8.  Neuronal lipidosis and neuroaxonal dystrophy in cerebro-hepato-renal (Zellweger) syndrome.

Authors:  J M Powers; R C Tummons; A B Moser; H W Moser; D S Huff; R I Kelley
Journal:  Acta Neuropathol       Date:  1987       Impact factor: 17.088

9.  Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy.

Authors:  W W Chen; P A Watkins; T Osumi; T Hashimoto; H W Moser
Journal:  Proc Natl Acad Sci U S A       Date:  1987-03       Impact factor: 11.205

10.  Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical and morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy.

Authors:  J Vamecq; J P Draye; F Van Hoof; J P Misson; P Evrard; G Verellen; H J Eyssen; J Van Eldere; R B Schutgens; R J Wanders
Journal:  Am J Pathol       Date:  1986-12       Impact factor: 4.307
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