Hepatic dysfunction in 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 کاملNovel Cell-Based Therapies in Hepatic Disorders
In the recent decade, liver diseases with high mortality rate have became one of the major health problems worldwide. Liver transplantation is the most effective and the standard treatment for decompensated liver disease, but, shortage of available organs and inaccuracy in modeling of the liver diseases are the most limiting issues in treatment. Regenerative medicine and cell based therapy str...
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...
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ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
سال: 2016
ISSN: 0167-4889
DOI: 10.1016/j.bbamcr.2015.09.035