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Titolo:
ABNORMALITIES OF GLYCOSPHINGOLIPID, SULFATIDE, AND CERAMIDE IN THE POLYCYSTIC (CPK CPK) MOUSE/
Autore:
DESHMUKH GD; RADIN NS; GATTONE VH; SHAYMAN JA;
Indirizzi:
UNIV MICHIGAN,DEPT INTERNAL MED ANN ARBOR MI 48109 UNIV MICHIGAN,DEPT INTERNAL MED ANN ARBOR MI 48109 UNIV KANSAS,MED CTR,DEPT ANAT & CELL BIOL KANSAS CITY KS 66105
Titolo Testata:
Journal of lipid research
fascicolo: 9, volume: 35, anno: 1994,
pagine: 1611 - 1618
SICI:
0022-2275(1994)35:9<1611:AOGSAC>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
GROWTH-FACTOR RECEPTOR; PROTEIN-KINASE-C; GANGLIOSIDE-MEDIATED MODULATION; KIDNEY-DISEASE EPITHELIA; NECROSIS-FACTOR-ALPHA; RENAL-CELL CARCINOMA; INOSITOL TRISPHOSPHATE; EXTRACELLULAR-MATRIX; MURINE MODEL; RAT-KIDNEY;
Keywords:
POLYCYSTIC KIDNEY; SULFOTRANSFERASE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
51
Recensione:
Indirizzi per estratti:
Citazione:
G.D. Deshmukh et al., "ABNORMALITIES OF GLYCOSPHINGOLIPID, SULFATIDE, AND CERAMIDE IN THE POLYCYSTIC (CPK CPK) MOUSE/", Journal of lipid research, 35(9), 1994, pp. 1611-1618

Abstract

Polycystic kidney disease is a disorder marked by aberrant renal tubular epithelial cell proliferation and transport abnormalities. Sphingolipids are ubiquitous membrane components implicated in several cellular functions including cell membrane sorting, signaling, growth, ion transport, and adhesion. To investigate a potential pathogenic role forsphingolipids in cystic kidney disease, we studied the sphingolipid content and associated enzymatic activities of the kidneys from cpk/cpkmice and their phenotypically normal litter mates. The neutral glycolipids, including glucosylceramide and lactosyl ceramide, displayed a striking increase in 3-week-old cpk/cpk mice as did the acidic lipid, ganglioside GM3. However, a correspondingly significant decrease in sulfoglycolipid and ceramide concentration was observed in the cpk/cpk kidneys. Glucosylceramide synthase activity was higher in the kidneys ofthe cpk/cpk mice than in those of the controls. Kinetic analysis of the glucosylceramide synthase revealed the presence of an endogenous activator in the cystic kidney. A marked decrease in sulfotransferase activity was observed in both whole kidney homogenates and in microsomalpreparations that was consistent with the decrement in sulfolipid content. The increase in GM3, glucosyl- and lactosylceramide may therefore be the result of impaired sulfolipid synthesis at the 3-week time point. While sulfolipid and glucosylceramide concentrations are not different at 1 and 2 weeks of age, ceramide concentrations in cystic kid neys are significantly reduced compared to kidneys from phenotypically normal mice. These results suggest that sphingolipids may play a potential role in the proliferative and transport abnormalities associated with cystic renal disease and the development of azotemia.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/11/20 alle ore 04:30:34