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Titolo:
Lysosome-related organelles
Autore:
DellAngelica, EC; Mullins, C; Caplan, S; Bonifacino, JS;
Indirizzi:
NICHHD, Cell Biol & Metab Branch, NIH, Bethesda, MD 20892 USA NICHHD Bethesda MD USA 20892 & Metab Branch, NIH, Bethesda, MD 20892 USA
Titolo Testata:
FASEB JOURNAL
fascicolo: 10, volume: 14, anno: 2000,
pagine: 1265 - 1278
SICI:
0892-6638(200007)14:10<1265:LO>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
HERMANSKY-PUDLAK-SYNDROME; CHEDIAK-HIGASHI-SYNDROME; BASOPHILIC LEUKEMIA-CELLS; CLASS-II COMPARTMENTS; AP-3 ADAPTER COMPLEX; TYROSINASE-RELATED PROTEIN; INTEGRAL MEMBRANE-PROTEIN; STORAGE POOL DEFICIENCY; DENSE GRANULE MEMBRANES; NATURAL-KILLER-CELLS;
Keywords:
melanosome; lytic granule; MIIC; platelet-dense granule; basophil granule; azurophil granule; pigment granule; Chediak-Higashi syndrome; Griscelli syndrome; Hermansky-Pudlak syndrome; protein trafficking;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
125
Recensione:
Indirizzi per estratti:
Indirizzo: Bonifacino, JS NICHHD, Cell Biol & Metab Branch, NIH, Bldg 18T,Room 101,18Lib Dr,MSC 5430, Bethesda, MD 20892 USA NICHHD Bldg 18T,Room 101,18 Lib Dr,MSC 5430 Bethesda MD USA 20892
Citazione:
E.C. Dell'Angelica et al., "Lysosome-related organelles", FASEB J, 14(10), 2000, pp. 1265-1278

Abstract

Lysosomes are membrane-bound cytoplasmic organelles involved in intracellular protein degradation, They contain an assortment of soluble acid-dependent hydrolases and a set of highly glycosylated integral membrane proteins, Most of the properties of lysosomes are shared with a group of cell type-specific compartments referred to as 'lysosome-related organelles', which include melanosomes, lytic granules, MHC class II compartments, platelet-densegranules, basophil granules, azurophil granules, and Drosophila pigment granules. In addition to lysosomal proteins, these organelles contain cell type-specific components that are responsible for their specialized functions. Abnormalities in both lysosomes and lysosome-related organelles have beenobserved in human genetic diseases such as the Chediak-Higashi and Hermansky-Pudlak syndromes, further demonstrating the close relationship between these organelles. Identification of genes mutated in these human diseases, as well as in mouse and Drosophila pigmentation mutants, is beginning to shed light on the molecular machinery involved in the biogenesis of lysosomes and lysosome-related organelles.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 22/09/20 alle ore 13:19:19