Neuroimaging has become an indispensable tool in understanding the regions of susceptibility , progression of disease, and therapeutic effectiveness for the management of neurologic diseases in humans. Metabolic diseases are recognized more frequently now as the biochemical basis is understood better. In addition, and as illustrated in this issue of the AJNR [1-3] , clinicians can benefit immen...

organic academia are diverse group of disorders characterized by abnormal degredation of specific amino acid in the specific catabolism due to an enzymeactivity defect which mainly due to enzyme deficiency. the majority of the classic organic acid disorders are caused by abnormal catabolism of branched-chain amino acids like lysine. maple syrup urine disease (msud), propionic acidemia, methylma...

Mutations in the gene encoding human ATP:cobalamin adenosyltransferase (hATR) can result in the metabolic disorder known as methylmalonic aciduria (MMA). This enzyme catalyzes the final step in the conversion of cyanocobalamin (vitamin B12) to the essential human cofactor adenosylcobalamin. Here we present the 2.5 A crystal structure of ATP bound to hATR refined to an Rfree value of 25.2%. The ...