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
Glycogen storage disease type Ia in canines: a model for human metabolic and genetic liver disease Specht A; Fiske L; Erger K; Cossette T; Verstegen J; Campbell-Thompson M; Struck MB; Lee YM; Chou JY; Byrne BJ; Correia CE; Mah CS; Weinstein DA; Conlon TJJ Biomed Biotechnol 2011[]; 2011 (ä): 646257A canine model of Glycogen storage disease type Ia (GSDIa) is described. Affected dogs are homozygous for a previously described M121I mutation resulting in a deficiency of glucose-6-phosphatase-alpha. Metabolic, clinicopathologic, pathologic, and clinical manifestations of GSDIa observed in this model are described and compared to those observed in humans. The canine model shows more complete recapitulation of the clinical manifestations seen in humans including "lactic acidosis", larger size, and longer lifespan compared to other animal models. Use of this model in preclinical trials of gene therapy is described and briefly compared to the murine model. Although the canine model offers a number of advantages for evaluating potential therapies for GSDIa, there are also some significant challenges involved in its use. Despite these challenges, the canine model of GSDIa should continue to provide valuable information about the potential for generating curative therapies for GSDIa as well as other genetic hepatic diseases.|*Disease Models, Animal[MESH]|Animals[MESH]|Clinical Trials as Topic[MESH]|Dog Diseases/*genetics/*metabolism[MESH]|Dogs[MESH]|Glycogen Storage Disease Type I/diagnosis/*genetics/*metabolism/pathology/veterinary[MESH]|Humans[MESH]|Liver Diseases/*genetics/*metabolism/veterinary[MESH] |
