William J. Zinnanti

Mouse model of encephalopathy and novel treatment strategies with substrate competition in glutaric aciduria type I (2010)

Zinnanti, William J., Lazovic, Jelena

Glutaric aciduria type I (GA-1) results from an inherited defect in a common step of lysine, hydroxylysine and tryptophan metabolism. This defect is associated with an age-dependent susceptibility to...

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease (2009)

Zinnanti, William J., Lazovic, Jelena, Griffin, Kathleen, Skvorak, Kristen J., Paul, Harbhajan S., Homanics, Gregg E., ...

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with lifethreatening cerebral oedema and dysmyelination in affected individuals. Treatment...

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease (2009)

Zinnanti, William J., Lazovic, Jelena, Griffin, Kathleen, Skvorak, Kristen J., Paul, Harbhajan S., Homanics, Gregg E., ...

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with life-threatening cerebral oedema and dysmyelination in affected individuals....

Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I (2007)

Zinnanti, William J., Lazovic, Jelena, Housman, Cathy, LaNoue, Kathryn, O'Callaghan, James P., Simpson, Ian, ...

Glutaric acidemia type I (GA-I) is an inherited disorder of lysine and tryptophan metabolism presenting with striatal lesions anatomically and symptomatically similar to Huntington disease. Affected...

A diet-induced mouse model for glutaric aciduria type I (2006)

Zinnanti, William J., Lazovic, Jelena, Wolpert, Ellen B., Antonetti, David A., Smith, Michael B., Connor, James R., ...

In the autosomal recessive human disease, glutaric aciduria type I (GA-1), glutaryl-CoA dehydrogenase (GCDH) deficiency disrupts the mitochondrial catabolism of lysine and tryptophan. Affected...

A diet-induced mouse model for glutaric aciduria type I (2006)

Zinnanti, William J., Lazovic, Jelena, Wolpert, Ellen B., Antonetti, David A., Smith, Michael B., Connor, James R., ...

In the autosomal recessive human disease, glutaric aciduria type I (GA-1), glutaryl-CoA dehydrogenase (GCDH) deficiency disrupts the mitochondrial catabolism of lysine and tryptophan. Affected...

A diet-induced mouse model for glutaric aciduria type I (2006)

Zinnanti, William J., Lazovic, Jelena, Wolpert, Ellen B., Antonetti, David A., Smith, Michael B., Connor, James R., ...

In the autosomal recessive human disease, glutaric aciduria type I (GA-1), glutaryl-CoA dehydrogenase (GCDH) deficiency disrupts the mitochondrial catabolism of lysine and tryptophan. Affected...

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease

Zinnanti, William J., Lazovic, Jelena, Griffin, Kathleen, Skvorak, Kristen J., Paul, Harbhajan S., Homanics, Gregg E., ...

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with life-threatening cerebral oedema and dysmyelination in affected individuals....