Warning: Undefined variable $zfal in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 525
Deprecated: str_replace(): Passing null to parameter #3 ($subject) of type array|string is deprecated in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 525
Warning: Undefined variable $sterm in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 530
free
Warning: Undefined variable $sterm in C:\Inetpub\vhosts\kidney.de\httpdocs\mlpefetch.php on line 531
free free
English Wikipedia
Nephropedia Template TP (
Twit Text
DeepDyve Pubget Overpricing |
lüll A novel role of the Batten disease gene CLN3: association with BMP synthesis Hobert JA; Dawson GBiochem Biophys Res Commun 2007[Jun]; 358 (1): 111-6Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) results from a deficiency of CLN3, a protein recently identified within detergent-resistant membranes (DRMs). To study the function of CLN3 within these domains we isolated DRMs from control and JNCL-brain and noted that JNCL-derived DRMs are less buoyant than control. Analysis of DRM phospholipids derived from JNCL-brain revealed a reduction of bis(monoacylglycerol)phosphate. Metabolic labeling of JNCL-fibroblasts demonstrated a reduction in the synthesis of bis(monoacylglycerol)phosphate which was restored following complementation with wild-type-CLN3, substantiating our initial observation in brain. Metabolic labeling of cell lines overexpressing wild-type-CLN3 resulted in increased bis(monoacylglycerol)phosphate synthesis, while overexpression of mutant CLN3-L170P decreased bis(monoacylglycerol)phosphate synthesis. These data illustrate a new finding, a strong correlation between CLN3 protein expression and synthesis of bis(monoacylglycerol)phosphate.|Brain/metabolism[MESH]|Cells, Cultured[MESH]|Fibroblasts/metabolism[MESH]|Humans[MESH]|Intracellular Membranes/metabolism[MESH]|Lysophospholipids/*biosynthesis[MESH]|Membrane Glycoproteins/genetics/*metabolism[MESH]|Membrane Microdomains/metabolism[MESH]|Molecular Chaperones/genetics/*metabolism[MESH]|Monoglycerides/*biosynthesis[MESH]|Mutation[MESH]|Neuronal Ceroid-Lipofuscinoses/*metabolism[MESH] |