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| DISORDERS OF PEROXISOME BIOGENESIS |
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| Author(s): BRAVERMAN N, DODT G, GOULD SJ, VALLE D |
| Source: HUMAN MOLECULAR GENETICS Volume: 4 Special Issue: Sp. Iss. SI Pages: 1791-1798 Published: 1995 |
| Times Cited: 41 References: 115 |
| Abstract: The peroxisome is a ubiquitous, subcellular organelle containing more than 50 matrix enzymes that participate in a diverse array of metabolic pathways. Failure to assemble normal peroxisomes is the cellular hallmark of Zellweger syndrome and other human disorders of peroxisome biogenesis. Identification of the genes required for peroxisome biogenesis is proceeding at a rapid pace helped immeasurably by work in other species, particularly various yeasts. The ultimate goals of this effort are to identify all of these genes and to understand how their protein products interact to produce normal appearing and functioning peroxisomes. Attainment of these goals will lead to a better understanding of the peroxisome biogenesis disorders, their pathophysiology and treatment. |
| Document Type: Review |
| Language: English |
Addresses:
1. JOHNS HOPKINS UNIV, SCH MED, DEPT PEDIAT, BALTIMORE, MD 21205 USA
2. JOHNS HOPKINS UNIV, SCH MED, DEPT BIOL CHEM, BALTIMORE, MD 21205 USA
3. JOHNS HOPKINS UNIV, SCH MED, DEPT CELL BIOL & ANAT, BALTIMORE, MD 21205 USA
4. JOHNS HOPKINS UNIV, SCH MED, HOWARD HUGHES MED INST, BALTIMORE, MD 21205 USA |
| Publisher: OXFORD UNIV PRESS UNITED KINGDOM, WALTON ST JOURNALS DEPT, OXFORD, ENGLAND OX2 6DP |
| Subject Category: Biochemistry & Molecular Biology; Genetics & Heredity |
| IDS Number: TC033 |
| ISSN: 0964-6906 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |