1992
DOI: 10.1001/archderm.128.9.1213
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Peroxisomal abnormality in fibroblasts from involved skin of CHILD syndrome. Case study and review of peroxisomal disorders in relation to skin disease

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Cited by 16 publications
(12 citation statements)
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“…However, lamellar inclusions were seldom found in the dermal fibroblasts. In the upper prickle layer, keratinocytes contained dense bodies with laminated membrane structure, but these are abnormal cementsomes and different from what was descdbed by Emami et al (8). With some reservation that tissue culture induces these abnormal structures or that there may be subtypes of CHILD syndrome, our tissue study does not support their peroxisome theory.…”
Section: Dermal Vs Epidermal Cause Of the Disease And Perojdsome Theorycontrasting
confidence: 91%
“…However, lamellar inclusions were seldom found in the dermal fibroblasts. In the upper prickle layer, keratinocytes contained dense bodies with laminated membrane structure, but these are abnormal cementsomes and different from what was descdbed by Emami et al (8). With some reservation that tissue culture induces these abnormal structures or that there may be subtypes of CHILD syndrome, our tissue study does not support their peroxisome theory.…”
Section: Dermal Vs Epidermal Cause Of the Disease And Perojdsome Theorycontrasting
confidence: 91%
“…Cells of lipid synthetic organs such as sebaceous glands and adrenal cortex are filled with mitochondria of variable ultrastructural morphology, including vesicular ones in the rat adrenal cortex. In the CHILD syndrome, in which peroxisome deficiency and oxidative degradation of prostaglandin Ej have been postulated (11), a large number of laminated structures are derived from abnormal cementsomes (12,13). although normal cementsomes were also present (12)(13)(14).…”
Section: Discussionmentioning
confidence: 99%
“…Deficient peroxisomal function in cultured fibroblasts has been described in both CDPX2 and CHILD syndromes (113,(117)(118)(119)(120) and in the murine homolog of EBP deficiency, the bare patches mouse (113), which displays cutaneous defects that, like the phenotype in CDPX2, resolve over time (113,121). The clinical phenotypes of the postsqualene sterologenesis and peroxisome biogenesis disorders bear some striking resemblances (121), including skeletal defects (chondrodysplasia punctata), central nervous system and/or hepatic involvement, and ichthyosis in the PEX7 disorders (rhizomelic chondrodysplasia punctata) and adult RD.…”
Section: Disorders Of Distal Sterologenesis With Ichthyosiform Phenotmentioning
confidence: 99%