Major Peroxisomal Membrane Polypeptides Are Synthesized in Cultured Skin Fibroblasts from Patients with Zellweger Syndrome
نویسندگان
چکیده
منابع مشابه
Peroxisomal organization in normal and cerebrohepatorenal (Zellweger) syndrome fibroblasts.
The reported absence of morphologically detectable peroxisomes in liver and kidney tissue cells from patients affected by the autosomic recessive, inherited metabolic disease known as cerebrohepatorenal, or Zellweger, syndrome was studied in fibroblasts, assuming it to be a generalized defect. Normal cultured fibroblasts were shown to contain peroxisomes according to morphological, biochemical,...
متن کاملPeroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders.
The peroxisomal oxidation of the long chain fatty acid palmitate (C16:0) and the very long chain fatty acids lignocerate (C24:0) and cerotate (C26:0) was studied in freshly prepared homogenates of cultured skin fibroblasts from control individuals and patients with peroxisomal disorders. The peroxisomal oxidation of the fatty acids is almost completely dependent on the addition of ATP, coenzyme...
متن کاملRole of Cultured Skin Fibroblasts in Aesthetic and Plastic Surgery
The skin is the largest tissue of man with several functions. Cosmetics/cosmeceuticals agents always are needed to be evaluated for their detrimental effects on the skin. Nowadays, the therapeutic potential of stem cells has and fibroblasts have increased the hope for a successful cell therapy in aesthetic medicine. Stem cells are unspecialized cells capable of renewing themselves through cell ...
متن کاملUltrastructural and cytochemical demonstration of peroxisomes in cultured fibroblasts from patients with peroxisomal deficiency disorders
The oxidation of very long chain fatty acids and synthesis of ether glycerolipids (plasmalogens) occurs mainly in peroxisomes. Zellweger's cerebrohepatorenal syndrome (CHRS) is a rare, inherited metabolic disease characterized by an apparent absence of peroxisomes, an accumulation of very long chain fatty acids, and a decrease of plasmalogens in tissues and cultured fibroblasts from these patie...
متن کاملIdentification of the Peroxisomal Biogenesis Factor 1 Gene Point Mutation in an Iranian Family with Zellweger Syndrome (ZS)
Background & Objective: Peroxisome biogenesis disorders (PBDs) are a group of diseases with peroxisomal dysfunction. Wide range of symptoms are associated with the disease which are due to mutations in the PEX genes. The PEX1 mutation occurs in Zellweger syndrome (ZS), a severe autosomal recessive condition with hypotonia, intellectual disability, and hepatic enlargement. The present study ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Pediatric Research
سال: 1989
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-198908000-00017