Peroxisomal Disorders
نویسندگان
چکیده
منابع مشابه
Peroxisomal disorders.
Peroxisomes are subcellular organelles catalyzing a number of indispensable functions in cellular metabolism. The importance of peroxisomes is stressed by the existence of an expanding number of genetic diseases in which there is an impairment of one or more peroxisomal functions. The prototype of this group of diseases is the cerebro-hepato-renal syndrome of Zellweger (ZS), first described as ...
متن کاملPeroxisomal disorders: the single peroxisomal enzyme deficiencies.
Peroxisomal disorders are a group of inherited diseases in man in which either peroxisome biogenesis or one or more peroxisomal functions are impaired. The peroxisomal disorders identified to date are usually classified in two groups including: (1) the disorders of peroxisome biogenesis, and (2) the single peroxisomal enzyme deficiencies. This review is focused on the second group of disorders,...
متن کاملPeroxisomes and peroxisomal disorders.
During their classic studies on the Separation of lysosomes by centrifugation of rat liver homogenates, De Duve and bis group (1) observed that certain oxidative enzymes, such äs Z)-amino acid oxidase and uric acid oxidase wbich produce hydrogen peroxide, and catalase which attacks hydrogen peroxide, were always found in a discrete fraction which could be separated from lysosomes and mitochondr...
متن کاملMolecular genetics of peroxisomal disorders.
Twenty five human peroxisomal disorders have been defined at this time. They are subdivided into two major categories: 1) the disorders of peroxisome biogenesis, in which the organelle fails to form normally, and there are defects that involve multiple peroxisomal functions; and 2) disorders that affect single peroxisomal enzymes. During the last five years the molecular defects have been ident...
متن کاملPeroxisomal disorders I: biochemistry and genetics of peroxisome biogenesis disorders.
The peroxisomal disorders represent a group of genetic diseases in humans in which there is an impairment in one or more peroxisomal functions. The peroxisomal disorders are usually subdivided into two subgroups including (i) the peroxisome biogenesis disorders (PBDs) and (ii) the single peroxisomal (enzyme-) protein deficiencies. The PBD group is comprised of four different disorders including...
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ژورنال
عنوان ژورنال: Pediatric Neurology Briefs
سال: 1988
ISSN: 2166-6482,1043-3155
DOI: 10.15844/pedneurbriefs-2-11-5